COVID-19 international experience in paediatric patients with congenital heart disease.

OBJECTIVE: As COVID-19 continues to affect the global population, it is crucial to study the impact of the disease in vulnerable populations. This study of a diverse, international cohort aims to provide timely, experiential data on the course of disease in paediatric patients with congenital heart disease (CHD). METHODS: Data were collected by capitalising on two pre-existing CHD registries, the International Quality Improvement Collaborative for Congenital Heart Disease: Improving Care in Low- and Middle-Income Countries and the Congenital Cardiac Catheterization Project on Outcomes. 35 participating sites reported data for all patients under 18 years of age with diagnosed CHD and known COVID-19 illness during 2020 identified at their institution. Patients were classified as low, moderate or high risk for moderate or severe COVID-19 illness based on patient anatomy, physiology and genetic syndrome using current published guidelines. Association of risk factors with hospitalisation and intensive care unit (ICU) level care were assessed. RESULTS: The study included 339 COVID-19 cases in paediatric patients with CHD from 35 sites worldwide. Of these cases, 84 patients (25%) required hospitalisation, and 40 (12%) required ICU care. Age <1 year, recent cardiac intervention, anatomical complexity, clinical cardiac status and overall risk were all significantly associated with need for hospitalisation and ICU admission. A multivariable model for ICU admission including clinical cardiac status and recent cardiac intervention produced a c-statistic of 0.86. CONCLUSIONS: These observational data suggest risk factors for hospitalisation related to COVID-19 in paediatric CHD include age, lower functional cardiac status and recent cardiac interventions. There is a need for further data to identify factors relevant to the care of patients with CHD who contract COVID-19 illness.

Predictive factors of recurrence after pediatric acute pericarditis

Abstract Objective The predisposing factors for pericarditis recurrence in the pediatric population have not yet been established. This study aimed to define the risk factors for the unfavorable prognosis of pediatric acute pericarditis. Methods This was a retrospective study that included all patients with acute pericarditis treated from 2011 to 2019 at a tertiary referent pediatric center. Results The study included 72 children. Recurrence was observed in 22.2% patients. Independent risk factors for recurrence were: erythrocyte sedimentation rate ≥ 50 mm/h (p = 0.003, OR 186.3), absence of myocarditis (p = 0.05, OR 15.2), C-reactive protein ≥ 125 mg/L (p = 0.04, OR 1.5), and non-idiopathic etiology pericarditis (p = 0.003, OR 1.3). Corticosteroid treatment in acute pericarditis was associated with a higher recurrence rate than treatment with non-steroid anti-inflammatory therapy (p = 0.04). Furthermore, patients treated with colchicine in the primary recurrence had lower recurrence rate and median number of repeated infections than those treated without colchicine (p = 0.04; p = 0.007, respectively). Conclusion Independent risk factors for recurrence are absence of myocarditis, non-idiopathic etiology pericarditis, C-reactive protein ≥ 125 mg/L, and erythrocyte sedimentation rate ≥ 50 mm/h. Acute pericarditis should be treated with non-steroid anti-inflammatory therapy. A combination of colchicine and non-steroid anti-inflammatory drugs could be recommended as the treatment of choice in recurrent pericarditis.

Predictive factors of recurrence after pediatric acute pericarditis.

OBJECTIVE: The predisposing factors for pericarditis recurrence in the pediatric population have not yet been established. This study aimed to define the risk factors for the unfavorable prognosis of pediatric acute pericarditis. METHODS: This was a retrospective study that included all patients with acute pericarditis treated from 2011 to 2019 at a tertiary referent pediatric center. RESULTS: The study included 72 children. Recurrence was observed in 22.2% patients. Independent risk factors for recurrence were: erythrocyte sedimentation rate≥50mm/h (p=0.003, OR 186.3), absence of myocarditis (p=0.05, OR 15.2), C-reactive protein≥125mg/L (p=0.04, OR 1.5), and non-idiopathic etiology pericarditis (p=0.003, OR 1.3). Corticosteroid treatment in acute pericarditis was associated with a higher recurrence rate than treatment with non-steroid anti-inflammatory therapy (p=0.04). Furthermore, patients treated with colchicine in the primary recurrence had lower recurrence rate and median number of repeated infections than those treated without colchicine (p=0.04; p=0.007, respectively). CONCLUSION: Independent risk factors for recurrence are absence of myocarditis, non-idiopathic etiology pericarditis, C-reactive protein≥125mg/L, and erythrocyte sedimentation rate≥50mm/h. Acute pericarditis should be treated with non-steroid anti-inflammatory therapy. A combination of colchicine and non-steroid anti-inflammatory drugs could be recommended as the treatment of choice in recurrent pericarditis.

Mediastinal teratoma presenting with respiratory distress and cardiogenic shock in a neonate.

Mediastinal teratomas are uncommon, fast-growing thoracic tumours, which are usually diagnosed in childhood or adolescence. Neonatal forms are the rarest and often present with life-threatening respiratory distress syndrome. In our case, respiratory failure in a neonate was aggravated by severe cardiogenic shock due to aortic and systemic venous compression, extreme heart displacement and rotation, necessitating an emergency operation on the first day of life.

Does Colchicine Substitute Corticosteroids in Treatment of Idiopathic and Viral Pediatric Pericarditis?

Background and Objectives: Recurrence of pericarditis (ROP) is an important complication of the acute pericarditis. The aim of this study was to analyse the influence of aetiology, clinical findings and treatment on the outcome of acute pericarditis. Methods: Data were retrospectively collected from medical records of patients treated from 2011 to 2019 at a tertiary referent heart paediatric center. Results: Our investigation included 56 children with idiopathic and viral pericarditis. Relapse was registered in 8/56 patients, 2/29 (7.41%) treated with nonsteroidal anti-inflammatory drugs (NSAID) and 6/27 (28.57%) treated with corticosteroids (CS) and NSAID. Independent risk factors for ROP were viral pericarditis (p = 0.01, OR 31.46), lack of myocardial affection (p = 0.03, OR 29.15), CS use (p = 0.02, OR 29.02) and ESR ≥ 50 mm/h (p = 0.03, OR 25.23). In 4/8 patients the first recurrence was treated with NSAID and colchicine, while treatment of 4/8 patients included CS. Children with ROP treated with CS had higher median number of recurrence (5, IQR: 2-15) than those treated with colchicine (0, IQR: 0-0.75). Conclusions: Independent risk factors for recurrence are CS treatment, viral aetiology, pericarditis only and ESR ≥ 50 mm/h. Acute pericarditis should be treated with NSAID. Colchicine and NSAID might be recommended in children with the first ROP.

Devastating Complications of Metal Strut Migration Following Pectus Excavatum Repair.

The modified Ravitch technique with metal struts and the Nuss operation have been the dominant operative techniques for treatment of pectus excavatum in the previous decades. We present devastating postoperative complications of a 16-year-old boy after the modified Ravitch procedure for a severe deformity utilizing two metal bars. Four months following surgery, one strut was removed after the displacement noted on a regular postoperative examination. Ten days after the strut removal, the patient complained of lower limb pain but the sensations were attributed to physical inactivity. Two months later, after pain intensification, the boy was diagnosed with bilateral arterial and venous lower limb thromboses and subsequently, the migration of the remaining metal strut intracardially with the free end in the left ventricular cavity embedded in massive thrombi. An urgent cardiac procedure was performed and the bar removed. Postoperatively, the boy made a full cardiac recovery but with severe neurological complications and subsequent death. Migration of metal struts is a rare complication and, except in our case, had been dealt with successfully. This case should emphasize more attention to the postoperative follow-up management of such patients.

Clinical implications of prenatal diagnosis of aorto-left ventricular tunnel on postnatal treatment and final outcome.

Kosutic J, Prijic S, Stajevic M, Kalaba M, Ninic S, Mikovic Z, Vujic A, Popovic S. Clinical implications of prenatal diagnosis of aorto-left ventricular tunnel on postnatal treatment and final outcome. Turk J Pediatr 2017; 59: 342-344. There are no more than 20 antenatally diagnosed aorto-left ventricular tunnel cases reported in the literature. In most of them the diagnosis was made indirectly and only after multiple fetal scans based on findings such as thick and dilated left ventricle and grossly dilated ascending aorta. We present a patient in whom a direct tunnel visualization and aorto-left ventricular tunnel diagnosis was made at the 30th gestation week after a single fetal scan using the recently introduced `cockade sign`. Clinical implications of antenatal diagnosis on postnatal treatment and outcome are also discussed.

Gastric tube esophageal reconstruction in children with esophageal atresia and caustic stricture Study of clinical value based on 25 single-center. Centre experience.

AIM: The aim of this study was estimation of clinical value of gastric tube esophagoplasty in children based on one single center experience. METHODS: Forty-nine patients with diagnosis of EA (n=22) and caustic esophageal injury (n=27) had undergone reverse gastric tube esophagoplasty during 25 years at our institution. Almost all EA patients received initially gastrostomy and cervical esophagostomy. Majority of CS patients had initially repeated dilatation therapy. Demographics, preoperative features (initial condition, initial treatment and nutritional status) and postoperative features (leakage, stenosis, nutritional status and disease-specific symptoms) were retrospectively reviewed and analyzed. Mean follow up time was 8.2 years. RESULTS: Of 49 patients, 2 patients died and gastric tube failed in one patient. The most common complications were anastomotic leak (52%) and anastomotic stricture (47,8%). Disease-specific symptoms (dysphagia and reflux symptoms) with important clinical manifestations were observed in 10.87% of patients. Preoperatively, all patients were eutrophic. One year after operation 96% of patients stayed eutrophic and two EA patients were malnourished. CONCLUSION: The most common complications such as anastomotic leak and anastomotic stricture may be considered benign. Postoperative nutrition, as good indicator of the procedure's adequacy, for the majority of patients is satisfactory. Gastric tube esophagoplasty is an appropriate alternative for esophageal replacement in children. KEY WORDS: Children, Gastric tube, Esophagoplasty.

Balloon dilation and surgical valvotomy comparison in non-critical congenital aortic valve stenosis.

Percutaneous balloon aortic valvoplasty (BAV) and surgical aortic valvotomy (SAV) are palliative procedures in patients with non-critical congenital valve stenosis. The aim of the study was to evaluate long-term BAV and SAV results after up to 24 years of follow-up. From 1987 to 2013, 74 consecutive interventions were performed in patients with aortic stenosis, and 62 were included in the study (39 BAVs and 23 SAVs). Age of BAV patients was 1.3 months to 17 years, and of SAV patients 1.2 months to 15 years. Although BAV patients were older, there was no difference between groups according to sex, valve function/morphology, and early/late follow-up results, with exception to hospitalization period. Significant pressure gradient reduction and aortic regurgitation increment were registered after procedures. Three patients did not survive early period after surgery. Follow-up period was 7.0 ± 5.4 and 9.0 ± 8.0 years after BAV and SAV, respectively (p = 0.242). Follow-up pressure gradient rose only in the BAV group, and was emphasized after 10-year-follow-up (p = 0.020). Significant aortic insufficiency progression was registered after 15 years of follow-up in both groups (p = 0.007 and p = 0.009, respectively). Mean reintervention-free survival was 12.0 years in the BAV and 14.5 years in the SAV group (p = 0.733), and mean survival without aortic valve replacement was 15.2 and 17.4 years, respectively (p = 0.877). BAV and SAV in patients with congenital aortic stenosis are very comparable in both early and late follow-up results.

Recurrent left atrial myxomas in Carney complex: a genetic cause of multiple strokes that can be prevented.

BACKGROUND: Intracardiac myxomas in Carney complex are significant causes of cardiovascular morbidity and mortality through embolic stroke and heart failure. The genetic, clinical, and laboratory characteristics of Carney complex-related strokes from atrial myxomas have not been described. The regulatory subunit (R1A) of the protein kinase gene (PRKAR1A) is mutated in >60% of patients with Carney complex. METHODS: We studied patients with strokes and cardiac myxomas that were hospitalized in our institution and elsewhere; a total of 7 patients with 16 recurrent atrial myxomas and >14 episodes of strokes were identified. RESULTS: Neurologic deficits were reported; in 1 patient, an aneurysm developed at the site of a previous stroke. All patients were females, were also diagnosed with Cushing syndrome, and all had additional tumors or other Carney complex manifestations. Other than gender, although there was a trend for patients being overweight and hypertensive, no other risk factors were identified. A total of 5 patients (71%) had a PRKAR1A mutation; all mutations (c418_419delCA, c.340delG/p.Val113fsX15, c.353_365del13/p.Ile118fsX6, c.491_492delTG/p.Val164fsX4, and c.177+1G>A) were located in exons 3 to 5 and introns 2 to 3, and all led to a non-sense PRKAR1A mRNA. CONCLUSIONS: Female patients with Carney complex appear to be at a high risk for recurrent atrial myxomas that lead to multiple strokes. Early identification of a female patient with Carney complex is of paramount importance for the early diagnosis of atrial myxomas and the prevention of strokes.

[Levosimendan treatment of severe acute congestive heart failure refractory to dobutamine/milrinone in children].

INTRODUCTION: Levosimendan is a novel positive inotropic agent which, improves myocardial contractility through its calcium-sensitizing action, without causing an increase in myocardial oxygen demand. Also, by opening ATP-sensitive potassium channels, it causes vasodilatation with the reduction in both afterload and preload. Because of the long half-life, its effects last for up 7 to 9 days after 24-hour infusion. CASE REPORT: We presented three patients 2, 15 and 17 years old. All the patients had severe acute deterioration of the previously diagnosed chronic heart failure (dilatative cardiomyopathy; univentricular heart with bidirectional Glenn anastomosis and restrictive bulboventricular foramen; bacterial endocarditis on artificial aortic valve with severe stenosis and regurgitation). Signs and symptoms of severe heart failure, cardiomegaly (cardio-thoracic index 0.65) and left ventricular dilatation (end-diastolic diameter z-score 2.6; 4.1 and 4.0) were confirmed on admission. Also, myocardial contractility was poor with ejection fraction (EF - 27%, 25%, 35%), fractional shortening (FS - 13%, 11%, 15%) and stroke volume (SV - 40, 60, 72 mL/m2). The treatment with standard intravenous inotropic agents resulted in no improvement but in clinical deterioration. Thus, standard intravenous inotropic support was stopped and levosimendan treatment was introduced. All the patients received a continuous 24-h infusion 0.1 microg/kg/min of levosimendan. In a single patient an initial loading dose of 11 microg/kg over 10 min was administrated, too. Levosimendan treatment resulted in both clinical and echocardiography improvement with the improved EF (42%, 34%, 44%), FS (21%, 16%, 22%) and SV (59, 82, 93 mL/m2). Hemodynamic improvement was registered too, with the reduction in heart rate in all the treated patients from 134-138 bpm before, to less than 120 bpm after the treatment. These parameters were followed by the normalization of lactate levels. Nevertheless, left ventricular end-diastolic diameter did not change after the levosimendan treatment. CONCLUSION: Our initial experience demonstrates that administration of levosimendan in patients with severe chronic heart failure not responsive to standard intravenous inotropic treatment might result in a significant clinical and hemodynamic improvement and that, in selected patients, it might be life saving. According to our best knowledge patients presented are the first pediatric patients treated with levosimendan in our country.

Early recurrent left atrial myxoma in a teenager with de novo mutation of Carney complex.

We report a case of an extremely early recurrence of left atrial myxoma in a 13-year-old girl. On hospital admission, the clinical presentation was of cerebral embolism with noticeable spotty skin pigmentation and hypertelorism. The left atrial myxoma originated from the roof of the left atrium. The histology specimen showed typical finding of a myxoma. Six months later a new intracardial mass was evacuated, the postoperative result showing the same type of myxomatous tissue. Genetic investigations demonstrated Carney complex. The genetic analysis of the child's family was negative, demonstrating de novo mutation of this rare disorder.

Costovertebral rib disarticulation and pleurectomy in Askin's tumor: a mandatory approach to radical surgery in children? Case report and guiedelines to surgical treatment.

The case report of Askin's tumor in a 16-year-old girl is focused on the still debatable surgical contg roversy in the treatment of PNET tumor, i.e., whether disarticulation of involved rib at the costovertabral joint should be accepted as the mandatory surgical procedure. It was concluded that the procedure, if feasible, may offer better prognosis of PNET because progression-free survival rate of patients without costovertebral junction involvement reported in multicenter studies was statistically significantly better than in patients in whom PNET has involved the costovertebral junction or bone metastases were present at the diagnosis. The cartilage is a natural barrier for tumor spread and this property should be augmented by radical surgery. Disarticulation of involved rib or ribs and pleurectomy should be routinely performed if the surgery is contemplated with proper timing between the cycles of induction chemotherapy.

Age-related role of ambulatory electrocardiographic monitoring in risk stratification of patients with complete congenital atrioventricular block.

AIMS: The aim of the paper was to assess the importance of 24 h electrocardiographic Holter monitoring in determining predictive factors for Adams-Stokes (AS) attacks and heart failure (HF) in children and adolescents with complete congenital atrioventricular block (CCAVB). METHODS AND RESULTS: Forty-five patients were divided into two groups according to the presence of AS attacks and HF and six age-related subgroups. The following parameters of 24 h electrocardiographic Holter monitoring were analysed: (i) minimum heart rate (HR), (ii) maximum HR, (iii) average HR, (iv) daytime HR (v) rhythm and conduction disturbance. Adams-Stokes attacks and HF occurred in 10 and 8 patients, respectively (40%). Five of six neonates with HF had maximum HR < 74 bpm and daytime HR < 58 bpm. Maximum HR below 68 bpm and daytime HR below 52 bpm were recorded in all the children up to 8 years of age with AS attacks and HF and only in 3 of 14 asymptomatic patients. All the patients above 8 years of age with AS attacks had maximum HR below 62 bpm. Of 6 patients with daytime HR < 50 bpm AS attacks were present in two. Episodes of marked ventricular slowing during sleep were registered in 4 of 10 (40%) patients and in 3 of 27 (11%) symptomless patients. CONCLUSION: Risk factors for development of AS attacks and HF in patients with CCAVB include: (i) maximum HR < 74 bpm in neonates, <68 bpm up to the age of 8 and <62 bpm at ages above 8, (ii) daytime HR <58 bpm in neonates and < 52 bpm till the age of 8, and (iiii) abrupt pauses in ventricular rate that are at least twice the basic cycle length after the neonatal period.

Echocardiographic analysis of the subtypes of right ventricular restrictive physiology in surgically treated patients with tetralogy of Fallot.

BACKGROUND: Our study focuses on echocardiographic assessment of the right ventricular diastolic function and adaptive right ventricular response to volume overload resulting from pulmonary and tricuspid regurgitation in surgically treated patients with tetralogy of Fallot. METHOD AND RESULTS: We included 60 patients subsequent to surgical correction of tetralogy of Fallot, dividing them into two groups - a group of 18 patients with restrictive physiology, having antegrade flow to the pulmonary arteries greater than 30 centimetres per second in late diastole in five consecutive beats, and a group of 42 patients with non-restrictive physiology. Based on the cardiothoracic ratio, being more or equal to, or less than 0.55, we further divided those with restrictive physiology into a group of 14 patients deemed to have primary restriction, and the other 4 patients considered to have secondary or acquired restriction. Those with non-restrictive physiology were divided into groups of 16 patients with a small heart, and 26 patients with a large heart. A fraction of the venous retrograde diastolic flow in the hepatic vein greater or equal to 30 centimetres is important for distinguishing between the subgroup with primary restriction and the other subgroups. In the four patients with secondary restriction, anterograde diastolic flow in the pulmonary artery greater than 30 centimetres per second was recorded after the average period of follow-up of 2.4 years. The mean value of the pulmonary regurgitant jet pressure half-time was higher in the subgroup with the secondary restriction in comparison to the nonrestrictive subgroup with large hearts at 152 milliseconds with standard deviation of 36 milliseconds versus 85 milliseconds with standard deviation of 11 milliseconds, p less than 0.05. This was significantly lower in comparison to those with primary restriction, where the value was 238 milliseconds, with standard deviation of 42 milliseconds, p less than 0.02. CONCLUSION: Echocardiographic analysis offers great possibilities for assessment of right ventricular diastolic function, identifying in particular those with restrictive physiology, its interrelation with pulmonary and tricuspid regurgitation, as well as timing and selection of patients for re-intervention.

Prevalence of venous obstruction in permanent endovenous pacing in newborns and infants: follow-up study.

OBJECTIVE: We examined the prevalence of venous obstruction in 12 newborns and infants with permanent endovenous ventricular pacing, clinically, and by ultrasonographic assessment of hemodynamics (spontaneity, phasicity, velocity, and turbulence of flow) and morphologic parameters (compressibility, wall thickness, and thrombus presence). MATERIAL AND METHODS: All implantations of single ventricular unipolar endovenous steroid leads, were performed via cephalic vein, and pacemakers were placed in subcutaneous pocket in right prepectoral region. After the vascular surgeon has carefully examined all children for presence of venous collaterals in the chest wall, morphologic and hemodynamic parameters of the subclavian, axillary, and internal jugular veins, were assessed by linear-array color Doppler. Lead capacity (LC) was calculated for each patient. RESULTS: Mean age of patients at implant was 6.2 months (range 1 day-12 months), mean weight 6.5 kg (range 2.25-10 kg), and mean height 60.9 cm (range 48-78 cm). Mean LC was 1.99 (range 1.14-3.07). Total follow-up was 1023 and mean follow-up 85.2 pacing months (range 3-156). No clinical signs of venous obstruction were observed. Mild stenosis (20%) of subclavian vein was found by color Doppler in 2/12 patients. Both had adequate lead diameter for body surface. CONCLUSION: Permanent endovenous pacing is a feasible procedure, even in children of body weight less than 10 kg, with quite acceptable impact on venous system patency.