Prenatal and Neonatal Pulmonary Thrombosis as a Potential Complication of SARS-CoV-2 Infection in Late Pregnancy.

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.

Genotype-phenotype correlation in two Polish neonates with alveolar capillary dysplasia.

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 12th 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 3rd 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.

A rare case of recurrent fetal goiter.

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.

Valve-sparing surgery for isolated cleft mitral valve complicated by endocarditis in a child.

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.

A biodegradable ring enables growth of the native tricuspid annulus.

BACKGROUND AND AIM OF THE STUDY: The study aim was to assess the growth potential and function of the tricuspid valve (TV) annulus after annuloplasty with the intra-annular biodegradable ring. METHODS: Among 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). RESULTS: The 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. CONCLUSION: The 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.

[Staphylococcal sepsis due to ventricular septal defect patch infection 14 years after its closure]. / Posocznica gronkowcow w wyniku zakazenia laty na ubytku przegrody miedzykomorowej 14 lat po jego zamknieciu.

We report the case of a 17 year-old girl who developed sepsis due to Methicillin-Resistant Staphyloccocus Epidermidis (MRSE) infection of Dacron patch 14 years after ventricular septal defect (VSD) closure and 4 years after pacemaker implantation. Although MRSE grew in many cultures taken and proper antibiotic regimen was administered, no improvement in patient's clinical status was observed. Disseminated intravascular coagulation and multi-organ failure developed. An operation, at which perforated Dacron VSD-patch was replaced with a new Gore-Tex one, was performed by cardiac surgery team. The patient's clinical status improved immediately after the procedure. The girl remained well 12 months after discharge.

[Closure of major aorto-pulmonary collateral artery with the Amplatzer vascular plug in an infant with pulmonary atresia and ventricular septal defect - a difficult therapeutic problem]. / Zamkniecie nieprawidlowego polaczenia aortalno-plucnego zatyczka Amplatzer vascular plug u dziecka z zarosnieta zastawka plucna i ubytkiem miedzykomorowym - trudny problem terapeutyczny.

A case of a 2-day-old newborn with congenital heart disease, pulmonary atresia and ventricular septal defect is described. The Blalock-Taussig shunt was created 6 days after birth. After the procedure, the newborn developed symptoms of increased pulmonary blood flow and respiratory distress. We decided to close one of the major aortopulmonary collateral arteries with the Gianturco coil on the 41st day after birth, however the results were not satisfactory. On the 66th day of infant life (by that time the patient weighted 3 kg), another procedure with the Amplatzer vascular plug was performed. This procedure was successful. After a few days the infant was discharged from hospital.

[24-hour pulse pressure in children with chronic kidney disease]. / Calodobowe cisnienie tetna u dziecl z przewlekla choroba nerek.

The aim of the study was to assess 24-h pulse pressure (PP) and to determine relationships between PP and echocardiographic parameters of left ventricle in children with chronic kidney disease (CKD). The study population included 47 children (mean age: 13.11 yrs) with CKD treated conservatively (n=14), with hemodialysis (HD) (n=13) and automated peritoneal dialysis (APD) (n=20). Retrospectively, antropometrical data, office blood pressure, ambulatory blood pressure monitoring (ABPM) variables and LV parameters in echocardiogaphy were analyzed. In study subjects, hypertension (HTN) was present in 25 (53.19%) and in 29 (61.7%) when based on office blood pressure and ABPM respectively. The prevalence of HTN was the highest in predialysis patients. 21 (44.6%) of children had left ventricular hypertrophy (LVH), which was the most frequently found in HD group. The mean PP value was 45.26 +/- 7.56 mmHg and was similar in all groups. Positive correlations were found between PP and weight (p < 0.001), height (p < 0.05), body surface area (p < 0.01) and LV measures (LV mass, LV posterior wall thickness and diastolic diameter of LV, all p < 0.05). Children with PP above median had tendency for greater LV mass (p = 0.06). There was no difference in severity of blood pressure and PP in children with/without LVH. However, in 45% of patients LVH was noted in whom the value of PP was greater than calculated normative value of PP (95 centile). HTN and LVH is common in children with CKD. A mean PP in children with CKD is lower when compared to adult population with CKD. The results confirm that PP increases with the age and an increasing PP has an influence on the changes of LV geometry. Assessment of PP in ABPM might help in identifying those with abnormal LV geometry.

The Usefulness of Magnetic Resonance Imaging of the Cardiovascular System in the Diagnostic Work-Up of Patients With Turner Syndrome.

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/m2 was present in six patients (17.1%) and above 2.5 cm/m2 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.

Late proximal pulmonary artery occlusion in a child with a single chamber after a right-sided Blalock-Taussig shunt.

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.

Aortoventriculoplasty and left ventricle function: long-term follow-up.

OBJECTIVE: Depressed cardiac function after aortoventriculoplasty is well known during the postoperative period. Little data exist concerning the long-term follow-up. The aim of this study is to determine whether septal incision has any permanent effect on the left ventricle function. METHODS: From 1988 to 2002, 45 patients received aortic mechanical prosthesis. These patients were divided into two groups. Group A consisted of 26 patients 5-18 years old, who underwent simple aortic valve replacement. Group B consisted of 19 patients 4-20 years old, who underwent the Konno procedure. Systolic and diastolic functions of the left ventricle were analyzed using echocardiography. For the systolic function, the following parameters were assessed: pressure gradient between left ventricle and ascending aorta, shortening and ejection fraction of the left ventricle. For the diastolic function, left ventricle-filling parameters were assessed: ratio of early to late filling velocity, deceleration slope of the early filling velocity and left ventricular isovolumetric relaxation time. Furthermore the percentage fraction of the aortic valve index (AOVI%) was calculated and compared between these two groups. RESULTS: After the surgery in group A, AOVI% dropped from 110+/-21 to 98+/-11%, while in group B it increased from 82+/-16 to 114+/-11%. As a result a higher residual pressure gradient across the aortic valve was noted in group A: 21.26+/-15 as compared to 11.17+/-5 mmHg in group B. A mean pressure above 30 mmHg appeared in group A 2 years after the surgery, while in group B this was obtained after 6 years. As for the diastolic function no significant difference was noted between these two groups. Overall there was one late death in group A, and in group B two early deaths, two reoperations because of excessive drainage and in two patients permanent pacemakers had to be implanted. CONCLUSIONS: Improvement of the systolic function after the surgery was noted in both groups. In patients with low AOVI%, postsurgical pressure gradient, either residual or recurrent, appeared during the follow-up. As for the septal incision, it may have some transient effects on the left ventricle function in the postoperative period, but no permanent sequelae were observed in the long-term follow-up.

Echocardiographic parameters of left ventricular systolic and diastolic function in infants, children and adolescents before and after surgical correction of secundum atrial septal defect.

BACKGROUND: Left ventricular (LV) dysfunction has been described in adults with secundum atrial septal defect (ASD II) and heart failure (CHF). This anomaly has been rarely regarded as a cause of CHF in pediatric patients with ASD II. AIM: To assess LV systolic and diastolic function in patients with ASD II before and after cardiosurgery as well as to establish the prognostic value of these parameters in infants, children and adolescents with ASD II. METHODS: LV systolic (LVEF) and diastolic function parameters (E/A, DCT, IVRT) were studied using Doppler echocardiography in 104 patients aged between 1-18 years with ASD II before cardiosurgery and 4 years afterwards. These parameters were compared with similar variables in 150 healthy infants, children and adolescents. RESULTS: Before surgery LVEF was significantly lower only in infants with ASD II and CHF when compared with healthy controls. Relaxation abnormalities of LV diastolic function were observed before surgery in 40.4% of patients, especially in infants and adolescents with CHF. These disturbances were still present in about 20% of patients after surgery, especially in infants. Before and after surgery both LV and RV end-diastolic volumes were normal in about 6-10% of patients with LV diastolic dysfunction. CONCLUSIONS: In patients with ASD II and CHF diastolic dysfunction of LV is more common than systolic dysfunction. RV volume overload mainly affects LV diastolic function but it is not the only cause of CHF in patients with ASD II, especially in infants and adolescents. After surgery, normalisation of LV diastolic function is not observed in about 20% of patients with ASD II, especially in those who had CHF before operation. Special monitoring of the circulatory system is necessary in patients with ASD II operated in infancy and adolescence.

[Influence of autonomic nervous system on electrolyte abnormalities in children with mitral valve prolapse]. / Wplyw autonomicznego ukladu nerwowego na wystepowanie zaburzen elektrolitowych u dzieci z wypadaniem platków zastawki dwudzielnej.

Measurement of the heart rate variability enables to assess the activity of the autonomous nervous system. The aim of the study was to evaluate the possible relationship between clinical status, serum sodium (Na), potassium (K), calcium (Ca), magnesium concentrations and HRV in children with mitral valve prolapse. The study group consisted of 151 children with MVP (age +/- SD: 12.2 +/- 3.1 years). Thirty patients out of the MVP group were symptomatic and 121 were asymptomatic. The total number of 165 healthy children (age +/- SD: 12.3 +/- 3.7 years) were included into the control group. We assessed power spectrum of HRV (low frequency component--LF, high frequency component--HF, and LF/HF ratio) from 5-minute recording during daytime and night. Serum Mg (p < 0.00001) and K (p < 0.03) concentrations were significantly lower in MVP group as compared to healthy children. A marked decrease in Mg concentration was observed in symptomatic children with MVP as compared to asymptomatic patients (p < 0.0001). A significant positive correlation was found between Mg concentration and HF and HF n.u. components (daytime and night) and significant negative correlation was observed between Mg concentration and LF and LF n.u. (daytime and night) parameters and LF/HF ratio (daytime and night). Symptomatic children with MVP had significantly higher LF n.u. and LF/HF ratio and a lower HF n.u. as compared to asymptomatic patients. The autonomic changes in MVP children (reduction in parasympathetic tone with sympathetic predominance) were associated with decreased serum Mg concentration and higher prevalence of clinical symptoms. Mg supplementation seems to be a prudent approach in symptomatic children with MVP.

[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.

Congenital heart defect with associated malformations in children.

BACKGROUND: Children with multisystem involvement including congenital heart defect (CHD) are a very salient problem. The purpose of this study was to evaluate the incidence of CHD associated with malformations of other systems and to assess the modalities of treatment and perioperative mortality among patients referred to the department of pediatric cardiac surgery. METHODS: The medical records of 1856 children were reviewed retrospectively from 1997 to 2002 to establish CHD and types of associated malformations. The connections between CHD and other lesions were investigated. Furthermore, the influence of patient and perioperative variables on mortality risk was scrutinized. Univariate and multivariate analyses were used. RESULTS: Eighty-four children (4.53%) had CHD and associated malformations. The malformations of digestive (35.7%), urinary (22.4%), and nervous (14.3%) systems were the most frequently observed associated defects. No relation was found between CHD and concomitant lesions. The results of multivariate logistic regression showed significant influence of patient age, primary cardiac procedure, and CHD type on mortality (ca 19%) in children with multiorgan lesions. CONCLUSIONS: The treatment of children with CHD and associated multiple lesions is connected with higher mortality risk. The following factors: younger age, urgency of surgical procedure, and primary surgical procedure had negative impact on patient's outcome. However, these risks in certain cases are inevitable. The cardiac procedure preceding the surgical operation may improve the overall effect of treatment because of circulatory stabilization, provided that the condition of the patient does not preclude any intervention at all.

A prospective study to determine the significance of ventricular late potentials in children with mitral valvar prolapse.

We aimed prospectively to determine the incidence of ventricular arrhythmias and ventricular late potentials in children with mitral valvar prolapse, and to assess whether signal-averaged electrocardiography could identify which such children were at high risk of developing ventricular tachycardia. In all, we examined 151 children with mitral valvar prolapse, at an age of 12.2 +/- 3.1 years, and 164 healthy subjects aged 12.3 +/- 3.7 years. All children underwent 24-hour ambulatory Holter monitoring and echocardiography. The children with mitral valvar prolapse were followed prospectively for a mean of 64 months. There was a significantly higher prevalence of ventricular arrhythmias in those with prolapse than in the controls (p < 0.0001). Runs of ventricular tachycardia were observed in 3 children with mitral valvar prolapse compared with one from the control group. Late potentials were more frequently observed in the children with mitral valvar prolapse than in those who were healthy (p < 0.0001), and also in those with prolapse suffering ventricular arrhythmias compared with those without ventricular arrhythmias (p < 0.02). During follow-up, 24 children with prolapsing mitral valves developed non-sustained ventricular tachycardia, giving a frequency of 3.1/100 subject-years. The sensitivity of late potentials was low, at 52%, for the identification of children with mitral valvar prolapse who developed ventricular tachycardia, although the specificity was high at 90%. This gave a positive predictive value of 50%, and a negative predictive value of 91%. We conclude that prolapse of the mitral valve predisposes to the development of ventricular arrhythmias and late potentials in children. An abnormal signal-averaged electrocardiogram is a specific, but not very sensitive, predictor for the development of ventricular tachycardia in such children.