Neonatal venous thrombosis is a rare condition that can be iatrogenic or occur due to viral infections or genetic mutations. Thromboembolic complications are also commonly observed as a result of SARS-CoV-2 infections. They can affect pediatric patients, especially the ones suffering from multisystem inflammatory syndrome in children (MIS-C) or multisystem inflammatory syndrome in neonates (MIS-N). The question remains whether the maternal SARS-CoV-2 infection during pregnancy can lead to thromboembolic complications in fetuses and neonates. We report on a patient born with an embolism in the arterial duct, left pulmonary artery, and pulmonary trunk, who presented several characteristic features of MIS-N, suspecting that the cause might have been the maternal SARS-CoV2 infection in late pregnancy. Multiple genetic and laboratory tests were performed. The neonate presented only with a positive result of IgG antibodies against SARS-CoV-2. He was treated with low molecular weight heparin. Subsequent echocardiographic tests showed that the embolism dissolved. More research is necessary to evaluate the possible neonatal complications of maternal SARS-CoV-2 infection.

Background Alveolar capillary dysplasia (ACD) is a rare cause of severe pulmonary hypertension and respiratory failure in neonates. The onset of ACD is usually preceded by a short asymptomatic period. The condition is refractory to all available therapies as it irreversibly affects development of the capillary bed in the lungs. The diagnosis of ACD is based on histopathological evaluation of lung biopsy or autopsy tissue or genetic testing of FOXF1 on chromosome 16q24.1. Here, we describe the first two Polish patients with ACD confirmed by histopathological and genetic examination. Case presentation The patients were term neonates with high Apgar scores in the first minutes of life. They both were diagnosed prenatally with heart defects. Additionally, the first patient presented with omphalocele. The neonate slightly deteriorated around 12 th hour of life, but underwent surgical repair of omphalocele followed by mechanical ventilation. Due to further deterioration, therapy included inhaled nitric oxide (iNO), inotropes and surfactant administration. The second patient was treated with prostaglandin E1 since birth due to suspicion of aortic coarctation (CoA). After ruling out CoA in the 3 rd day of life, infusion of prostaglandin E1 was discountinued and immediately patient’s condition worsened. Subsequent treatment included re-administration of prostaglandin E1, iNO and mechanical ventilation. Both patients presented with transient improvement after application of iNO, but died despite maximized therapy. They were histopathologically diagnosed post-mortem with ACD. Array comparative genomic hybridization in patient one and patient two revealed copy-number variant (CNV) deletions, respectively, ~ 1.45 Mb in size involving FOXF1 and an ~ 0.7 Mb in size involving FOXF1 enhancer and leaving FOXF1 intact. Conclusions Both patients presented with a distinct course of ACD, extra-pulmonary manifestations and response to medications. Surgery and ceasing of prostaglandin E1 infusion should be considered as potential causes of this variability. We further highlight the necessity of thorough genetic testing and histopathological examination and propose immunostaining for CD31 and CD34 to facilitate the diagnostic process for better management of infants with ACD.

Cardiovascular defects occur in 50% of patients with Turner syndrome (TS). The aim of the study was to estimate the usefulness of cardiac magnetic resonance imaging (CMR) and magnetic resonance angiography (angio-MR) as diagnostics in children and adolescents with TS. Forty-one females with TS, aged 13.9 +/- 2.2 years, were studied. CMR was performed in 39 patients and angio-MR in 36. Echocardiography was performed in all patients. The most frequent anomalies diagnosed on CMR and angio-MR were as follows: elongation of the ascending aorta (AA) and aortic arch, present in 16 patients (45.7%), a bicuspid aortic valve (BAV), present in 16 patients (41.0%), and partial anomalous pulmonary venous return (PAPVR), present in six patients (17.1%). Aortic dilatation (Z-score > 2) was mostly seen at the sinotubular junction (STJ) (15 patients; 42.8%), the AA (15 patients; 42.8%), the thoracoabdominal aorta at the level of a diaphragm (15 patients; 42.8%), and the transverse segment (14 patients; 40.0%). An aortic size index (ASI) above 2.0 cm/m(2) was present in six patients (17.1%) and above 2.5 cm/m(2) in three patients (8.6%). The left ventricular end-diastolic volume (EDV), end-systolic volume (ESV), and stroke volume (SV) were diminished (Z-score< -2) in 10 (25.6%), 9 (23.1%), and 8 patients (20.5%), respectively. A webbed neck was correlated with the presence of vascular anomalies (p = 0.006). The age and body mass index (BMI) were correlated with the diameter of the aorta. Patients with BAV had a greater aortic diameter at the ascending aorta (AA) segment (p = 0.026) than other patients. ASI was correlated with aortic diameter and descending aortic diameter (AD/DD) ratio (p = 0.002; r = 0.49). There was a significant correlation between the right ventricular (p = 0.002, r = 0.46) and aortic diameters at the STJ segment (p= 0.0047, r = 0.48), as measured by echocardiography and CMR. Magnetic resonance can identify cardiovascular anomalies, dilatation of the aorta, pericardial fluid, and functional impairment of the ventricles not detected by echocardiography. BMI, age, BAV, and elongation of the AA influence aortic dilatation. The ASI and AD/DD ratio are important markers of aortic dilatation. The performed diagnostics did not indicate a negative influence of GH treatment on the cardiovascular system.

The paper presents the management of a child born with pulmonary valve atresia, a single (double-inlet) ventricle, right ventricular hypoplasia, and perimembranous septal defect. The first stage of treatment consisted in a Blalock-Taussig shunt. Control angiography performed 1 year after surgery confirmed that the anastomosis was correct, and there was no narrowing at the connection. The first stage of treatment was complicated by the occlusion of the left pulmonary artery, as diagnosed during cardiac catheterization before the planned bidirectional Glenn anastomosis. A decision was made to perform surgery through a left thoracotomy without a cardiopulmonary bypass in order to restore the continuity of the left pulmonary artery.

Congenital heart defects are found in 50% of girls with Turner syndrome (TS). The evaluation of cardiovascular system is therefore an important element in the diagnostic work-up of TS and is of particular significance of further cardiologic monitoring, safety aspects of recombinant growth hormone treatment (rGH) and any pregnancy planning. Monika Obara-Moszynska Szymon Rozmiarek Magdalena Lanocha Anna Kociemba Barbara Rabska-Pietrzak 1 Magdalena Janus Andrzej Siniawski Bartlomiej Mrozinski Marek Niedziela Malgorzata Pyda

We report a case of a female neonate with an incomplete (Class II) pentalogy of Cantrell (PC) presenting: omphalocoele, thoracoabdominal type of partial ectopia cordis with ventricular septal defect and valvular pulmonary stenosis. The patient underwent a successful complete operation. We discuss associated anomalies that might occur with PC and the general overall prognosis for patients with PC. This report describes a very rare case of a patient with PC and coexisting partial ectopia cordis who survived.

Introduction. Coarctation of the aorta (CoA) is a congenital narrowing of the aorta at the aortic isthmus. Computed tomography angiography (CTA) plays a major role in the imaging of aortic pathologies in children. As a non-invasive method, CTA combined with echocardiography is increasingly used for precise evaluation of cardiovascular pathologies. The aim of the study was to evaluate the use of CTA in children with ambiguous echocardiographic diagnosis of CoA or in patients before planned surgical repair of an aortic arch defect, and to evaluate the agreement between measurements of the aortic dimensions by CTA and echocardiography. Material and methods. We retrospectively evaluated CTA studies performed in 37 children with suspected CoA and compared aortic diameter measurements by CTA and echocardiography. In all patients, the effective dose was estimated using CTA protocol data.

Introduction. Coarctation of the aorta (CoA) is a congenital narrowing of the aorta at the aortic isthmus. Computed tomography angiography (CTA) plays a major role in the imaging of aortic pathologies in children. As a non-invasive method, CTA combined with echocardiography is increasingly used for precise evaluation of cardiovascular pathologies. The aim of the study was to evaluate the use of CTA in children with ambiguous echocardiographic diagnosis of CoA or in patients before planned surgical repair of an aortic arch defect, and to evaluate the agreement between measurements of the aortic dimensions by CTA and echocardiography. Material and methods. We retrospectively evaluated CTA studies performed in 37 children with suspected CoA and compared aortic diameter measurements by CTA and echocardiography. In all patients, the effective dose was estimated using CTA protocol data. Results. Based on CTA findings, CoA was diagnosed in 26 children, including 5 children with simple CoA, while 21 children had complex CoA with other coexisting anomalies. A number of additional pathologies were identified when evaluating other chest structures. Statistical analysis showed the agreement of aortic diameter measurements by the two imaging methods. The mean effective dose was 7.5 mSv. Conclusions. CTA allows precise imaging of aortic pathology in children and is an important diagnostic tool. Echocardiography remains the primary tool for imaging of CoA but has some limitations, particularly in older patients. Indications for CTA should be carefully considered due to the associated radiation exposure.

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.

3D non-fluoroscopic reconstruction of the heart with Ensite/NavX encourage to exclude radiation which is potentially harmful in paediatric population. The exclusion of x-rays may prolong the procedure or be related to lower success rate. The aim of the prospective study was to assess the duration of all steps of the procedure, fluoroscopy time, and radiation and success rate for fluoroscopic and Ensite groups. All patients undergoing EPS were included to the study. Procedure was performed using 2-electrode approach for all supraventricular arrhythmia. In VT, only ablation electrode was used. In Ensite group procedure was started from RA reconstruction, followed by CS, tricuspid valve and His bundle. Left sided WPW ablation was performed retrogradely or transseptally. Aorta was reconstructed before passage to left ventricle. Short fluoroscopy was always used during transseptal puncture, when VT originated close to aortic valve to tag LM and RCA or when CS diverticulum was suspected. The study group included 362 patients (age 14±5 years). There were 21 diagnostic EPS and 89 patients with AVNRT, 206 with WPW, 17 with atrial tachycardia and 28 with ventricular arrhythmia. 247 patients underwent fluoroscopic procedure, and in 115 patients Ensite NavX was used. Ensite reduced significantly the fluoro time and radiation (3,4±6,1 vs 16,6±13,7 min, 20±58mGy vs 58±95 mGy) and decreased the procedure time (69±30min vs 77±30min, p=0.001). The duration of 3D reconstruction was 15,7±11,5 min, preablation EPS 13,6±10 min, and ablation with postablation evaluation 28±23min. In the NavX group "0" fluoroscopy ("0-fluoro") ablation was achieved in 61 (53%) patients, and in 77 (67%) patients fluoro-time was below 1 min. The x-ray was used for verification of ablation electrode position, coronary angiography, CS angiography, transseptal puncture or unstable Ensite behavior. In AVNRT group Ensite significantly reduced the fluoroscopy (10,0±6,9 vs 0,8±2,7 min) and in 80% ablation was "0-fluoro". In WPW patients Ensite significantly reduced fluoroscopy (18,8±13,7 vs 4,4±6,5 min) and in 50% ablation was "0-fluoro". In transseptal technique (n=30) the Ensite reduced all analyzed parameters and there were no difference between transseptal and transaortic procedure. Ablation failed in 5,8% if Ensite group and in 6,4% of fluoro group. The introduction of non-fluoroscopic navigation reduces the x-ray exposition without increasing the procedure time nor affecting success rate. The "0" fluoroscopy ablation can be achieved in most of right-sided arrhythmia, while more technology is required for left sided substrates.

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.

In case of significant hypoxia in the course of congenital heart defect, when definite surgery is difficult, various palliative procedures are performed. One of the most frequently used is a modified Blalock-Taussig (BT) anastomosis. The BT anastomosis is closed during cardiac surgery. In some patients, BT anastomosis remains open after the surgery and requires closure by cardiac intervention using different types of closure devices. We describe a case of percutaneous closure of Blalock-Taussig anastomosis in a 4-year-old boy, with occluded left subclavian artery, presence of collateral circulation, and the subclavian artery steal syndrome to the left pulmonary artery through the patent BT anastomosis.

We report the case of fetal goiter which occurred in two consecutive pregnancies in the same patients. The first one, due to too late diagnosis and no intrauterine treatment, contributed to the immediate postnatal death of the newborn; the second one was properly diagnosed at 19 weeks and then effectively treated prenatally which allowed to avoid the fatal complications for the fetus and the newborn.

BACKGROUND AND AIM OF THE STUDYThe study aim was to assess the growth potential and function of the tricuspid valve (TV) annulus after annuloplasty with the intra-annular biodegradable ring.METHODSAmong 11 children (median age 4.5 years; age range: 0.2-10.9 years) who underwent surgery for severe congenital TV regurgitation, valve repair was completed using a biodegradable annuloplasty ring. The children were followed regularly using transthoracic echocardiography, whereby the TV annulus lateral diameter (TVALD) and valve function were monitored. Rates of valve growth were derived from the slope of the regression equations which related TVALD to the natural logarithm of the body surface area (lnBSA).RESULTSThe children's somatic growth was harmonious throughout the entire follow up period (mean 478 days; range: 171-1,477 days). The TVALD differed significantly at six months and at one and two years after surgery compared to the postoperative value at discharge, rising from 19 mm (range: 15.5-26.0 mm) to 24 mm (range: 19.0-30.0 mm) at the last control examination (p = 0.003), while the related Z-scores remained stable. A significant linear correlation between TVALD and lnBSA was found in 63.6% of patients. The median rate of growth for the whole cohort was 1.96-fold (range: 0.52-5.53-fold) higher than a norm, and correlated strongly and positively with age (r = 0.91; p <0.05). The median postoperative TV insufficiency fraction of 9.8% (range: 0-28.8%) remained constant during the follow up period. The postoperative TV maximal pressure gradient was 5.5 mmHg (range: 3.1-12.2 mmHg), and did not increase over time.CONCLUSIONThe implantation of a biodegradable ring does not restrict growth of the native TV annulus; this enabled its stabilization in proportion to the somatic growth in the majority of the children. The TV annulus began to change its dimension at six months postoperatively, which may coincide with its biodegradation. The rate of growth of the TV annulus differed from that in the normal population, and was proportional to the patient age. The increase in TV annulus diameter over time did not have any negative influence on the function of the repaired valve.

A 12-year-old girl presented with an isolated cleft mitral valve complicated by infective endocarditis that was confined to the medial part of the cleft anterior leaflet. An innovative valve-sparing surgery was applied whereby the destroyed part of the leaflet was removed. The corresponding anterior annulus was plicated by approximation of the base of the cleft to the posteromedial commissure. The 'cleft' edge of AML was then sutured to the plicated annulus and to P3 at the level of the posteromedial commissure. The reconstructed valve was fully functional, and showed trivial regurgitation and an absence of stenosis for up to two years postoperatively.