Twelve Years of Complete Atrioventricular Septal Defect Repair.

BACKGROUND: Surgical repair is the standard treatment for complete atrioventricular septal defect. At our institution, this repair is performed by single patch, modified single patch or two patch techniques, according to the surgeon preferences and the surgical anatomy of the defect. The goal of this study was to evaluate our results from the last twelve years. METHODS: From June 2006 to June 2018, 81 children with complete atrioventricular septal defect (without tetralogy of Fallot or unbalanced ventricles) were submitted to surgical repair at our institution. Data from all patients was retrospectively collected and evaluated. RESULTS: The average age was 6.9 ± 13.7 months and 84% had Down syndrome. Eighty percent were symptomatic and 6 patients were previously submitted to pulmonary artery banding. No more that mild left atrioventricular valve insufficiency was found in 84% and 89% of the patients, at discharge and follow-up, respectively. Small residual septal defects were present in 27% at discharge; during follow-up, 41% of these closed spontaneously. Pulmonary hypertension at discharge and follow-up appeared in 3.7% and 1.3%, respectively. Permanente pacemaker was implanted in 3 patients. Left ventricle outflow tract obstruction was found in 3 patients and 2 needed surgical correction. At follow-up (40 ± 38 months), 90% of the patients presented NYHA functional class I. No significant differences in the main repair outcomes were found between techniques, with the exception of small residual septal defects, although the groups were unmatched. CONCLUSIONS: Overall and regardless of the technique used for the repair of complete AVSD, good early and midterm outcomes were achieved.

First-in-man pulmonary artery stenting in children using the Bentley® BeGrow™ stent system for newborns and infants.

BACKGROUND: Stent implantation into growing vessels is a common treatment option in infants and children with congenital heart disease (CHD) and corresponding vessel lesions. After stent implantation in small children, repetitive stent redilations are frequently necessary to accommodate for somatic growth. Until now, all available stents have limited final expansion diameters. MATERIAL AND RESULTS: The new Bentley BeGrow™ stent system for newborns and infants is a L605 cobalt­chromium, pre-mounted, balloon expandable stent, which is compatible with a 4 French sheath and 0.014 inch guide wire thus allowing implantation in small vessels (4-6 mm). It offers a new, unique stent design that allows post-dilation steps up to Ø11.5 mm. While re-dilating up to Ø11.5 mm this new stent maintains radial force and shows uniform expansion with only minimal foreshortening. Predetermined breaking points allow the stent struts to break in a controlled manner when exceeding a diameter of 11.5 mm. Residual radial force maintains even after stent opening due to spiral arrangement of the predetermined breaking points. The 2 first-in-man pulmonary artery stent implantations in a newborn with univentricular circulation and a toddler with biventricular circulation are reported as part of the currently performed licencing trial (ClinicalTrials.govNCT03287024). CONCLUSION: The low-profile BeGrow™ stent system offers new treatment options for transcatheter stent implantations in newborns and infants. In our first experience, it can be effectively implanted. Longer follow-up will evaluate multiple, stepwise redilations and controlled stent strut breakage, which have the potential to accommodate for somatic vessel growth and/or subsequent implantation of larger stents.

Feasibility, safety and diagnostic impact of endomyocardial biopsies for the diagnosis of myocardial disease in children and adolescents.

AIMS: Endomyocardial biopsies (EMBs) are performed infrequently in children owing to significant past complication rates and controversial discussions about the therapeutic value of results. The objective of this study was to investigate the safety and feasibility of EMBs for suspected myocardial disease in relation to their clinical value. METHODS AND RESULTS: We performed a retrospective multicentre review of the Working Group for Interventional Cardiology of the German Society for Paediatric Cardiology. During three consecutive years, 206 EMBs (84 female/mean age 8.95±6.62 years) were performed and analysed at 15 heart centres. In the majority of cases, biopsies were taken from the right ventricle (RV/89.8%; p<0.001). The overall complication rate was 9.7%, whereas major complications occurred in only 0.97% of cases. Risk factors associated with a higher complication rate were biopsy during the first year of life (20.5%) and from the left ventricle (31.1%) (p<0.05). There was no procedure-related mortality. Treatment was changed in 18.0% of cases based on biopsy results. CONCLUSIONS: Today, endomyocardial biopsies in older children with suspected myocardial disease can be performed safely with a low risk of major complications and mortality, whereas the risk of complications if the biopsy is carried out in the first year of life or taken from the left ventricle remains high.

Unnoticed aspiration of palate plate impression material in a neonate: Diagnosis, therapy, outcome.

A 7 week old infant was admitted with respiratory failure. Chest X-ray showed an atelectatic right upper lobe, herniation of right middle and lower lobe into the left thorax, and compression-atelectasis of the left lung. Thoracic CT showed complete occlusion of the right superior bronchus with a radiopaque foreign material. Six weeks prior, an impression for a palate plate using polyvinylsiloxane was taken. The material was removed with a rigid endoscopy, in readiness of extracorporeal oxygenation in case of tracheal obstruction or bronchial rupture. After 4 months of follow-up a hyper reactive airway with recurrent upper and lower airway infections remains.

A Novel Designed Valved Conduit for RVOT Reconstruction in Grown-up Congenital Heart Patients: a Glimpse Down the Road.

BACKGROUND: A plethora of valves and valve conduits are available for reconstruction of the right ventricular outflow tract (RVOT) for grown-up congenital heart patients. However, for several reasons, the ideal pulmonary valve substitute still remains the subject of debate. In this study, we investigated the preliminary clinical and echocardiographic results after implantation of the RVOT Elan (Vascutek, Renfrewshire, United Kingdom) conduit in adolescents and adults. MATERIAL AND METHODS: Between October 2012 and December 2014, a total of 27 patients (19 males, mean age: 23.7 ± 22.5; range: 9-74 years) received a RVOT Elan conduit for RVOT reconstruction and were prospectively followed up clinically and echocardiographically. Twenty-five patients had previous cardiac surgery. The median number of prior operations per patient was 2 (range: 1-4). Tetralogy of Fallot was the most common diagnosis (n = 7). RESULTS: At a mean follow-up time of 0.9 ± 0.61 years (100% complete), all patients (27 of 27) were alive and in New York Heart Association Class I. Adverse events defined as valve failure, thrombosis, embolism, bleeding, or endocarditis did not occur. Freedom from reoperation in general was 100%. At 1-year follow-up, median peak pressure gradients (Δ Pmax) across the RVOT Elan conduit were 15 ± 3.2; 15.3 ± 2.1Δ, 16 ± 4.8, and 16.3 ± 5.1 mm Hg for the 19 (n = 3), 21 (n = 3), 23 (n = 6), and 25 mm (n = 15) conduit size, respectively. CONCLUSION: The RVOT Elan conduit revealed excellent preliminary clinical and hemodynamic performances independent from the underlying cardiac pathology with insignificant transvalvular gradients and nonturbulent flow characteristics.

Valve-sparing reimplantation technique for treatment of neoaortic root dilatation late after the arterial switch operation: raising the bar.

Neoaortic root dilatation can develop during long-term follow-up after an arterial switch operation (ASO). Although few patients require surgical reintervention, significant valve regurgitation is still an important cause of late morbidity. We report on a 15-year-old boy with significant dilatation of the neoaortic root that was treated with the valve-sparing reimplantation technique. There is only one reported case of valve-preserving surgery late after the ASO. Valve preservation is believed to be superior to valve replacement in patients with aortic regurgitation due to better hemodynamic performance and avoidance of anticoagulation therapy.

Life-threatening disseminated tuberculosis as a complication of TNF-α blockade in an adolescent.

Life-threatening disseminated tuberculosis developed in a 17-year-old girl who was treated with the TNF-α blocker adalimumab for refractory SAPHO syndrome. The patient presented to the emergency department with dyspnea and somnolence and within 2 h developed the clinical picture of a septic shock. In addition to this unusual presentation, she showed a complicated course with increasing cerebral granuloma formation in spite of adequate antimycobacterial treatment. Immune reconstitution after discontinuation of TNF blockade may contribute to this "paradoxical reaction." Possible implications for screening, diagnosis, and treatment of tuberculosis in children and adolescents receiving anti-TNF treatment are discussed.

Successful treatment of atrial flutter with amiodarone in a premature neonate. Case report and literature review.

This case report describes a 30-week gestation neonate who presented at birth with hydrops fetalis due to atrial flutter. Digoxin and electric cardioversion were unsuccessful in maintaining a stable sinus rhythm. The infant continued with intractable atrial flutter and severe hemodynamic deterioration until intravenous loading of amiodarone achieved conversion to stable sinus rhythm. Amiodarone was continued for 45 days; there was no recurrence of atrial flutter. Of note, the infant developed severe chronic lung disease after mechanical ventilation for 28 days. A lung biopsy ruled out amiodarone-induced pulmonary toxicity. A table is provided reviewing the different forms of neonatal supraventricular tachycardias. Apart from the successful management of the tachycardia, the role of amiodarone as an effective antiarrhythmic agent and its potential side effects, such as pulmonary toxicity and transient hypothyroidism, are discussed.

Autonomic function testing in children and adolescents with diabetes mellitus.

Cardiac autonomic neuropathy (CAN) is a common complication in type 1 diabetes mellitus (T1DM) and associated with an increased mortality. Early detection of CAN would be desirable for a better individual risk stratification. The aim of this study was to determine whether autonomic dysfunction can be diagnosed in young patients with a recent history of T1DM. Autonomic function was assessed in 20 pediatric patients with T1DM, aged 10-19 yr, and a control group of 136 non-diabetic patients using four cardiorespiratory reflexes: heart rate and blood pressure response in standing position, deep breathing, and Valsalva maneuver. Furthermore, power spectral analyses of the low- and high-frequency band of heart rate variability (HRV) and baroreflex sensitivity (BRS) were tested with the non-invasive Task force monitor (CNSystems, Graz, Austria). Cardiorespiratory reflexes were pathologic for at least one item in 75% of the diabetic and 60% in the healthy control group. A reduced BRS was always combined with abnormal HRV. We found this pattern in 30% of diabetic patients and never in the control group. In patients with impaired BRS, mean hemoglobin A1c (HbA1c) was 7.7% and duration of diabetes 6.5 yr. This did not differ from the overall value of the diabetic group: HbA1c level 8.4% and diabetes duration 7.3 yr. In conclusion, signs of autonomic dysfunction are not uncommon in an early stage of diabetes in young patients. Classical cardiorespiratory reflexes seem to be less specific than HRV and BRS as testing methods.

Common patterns of response to the head-up tilt test in children and adolescents.

Testing using the head-up tilt table is performed regularly as a diagnostic tool in the evaluation of syncope. Recommendations for protocols, and interpretation of the results, however, are mainly based on experience in adults. We evaluated the results of tilt table testing in 100 consecutive children and adolescents aged from 6 to 18 years and referred for investigation of syncope. Over half the patients, 55%, proved impossible to classify using the criterions established by the European Society of Cardiology. Based on our data, we propose a modified classification for responses to tilt table testing in the young.

Patent foramen ovale using the Premere device: the results of the CLOSEUP trial.

OBJECTIVES: The CLOSEUP trial was conducted to determine the safety and effectiveness of the Premere closure device in closure of patent foramen ovale (PFO). BACKGROUND: PFO is a relatively common congenital condition, associated with cryptogenic stroke and migraine with aura. The Premere device is specifically designed to close PFO of variable size and length, with right and left anchor arms connected by a flexible tether. The device has an open architecture, a low profile, and a small surface area on the left atrial side which may discourage thrombus formation. METHODS: Patients between 18 and 65 years of age who had a cryptogenic ischemic stroke or a transient ischemic attack and a PFO underwent percutaneous PFO closure using the Premere device. RESULTS: Of the 73 enrolled patients, six patients had atrial anatomy not appropriate for the Premere; 27 patients received the 15 mm and 40 patients received the 20 mm device. Implantation was successful in all patients. At 6 months of follow-up, 86% of patients had no shunt that could be provoked with Valsalva as assessed during contrast echocardiography. Closure rates were better with the 20 mm versus the 15 mm device, and three patients with residual shunt had atrial septal aneurysms at baseline. One patient had transient atrial fibrillation which resolved by 3 months. There were no instances of thrombus, death, or stroke. CONCLUSIONS: These data demonstrate that the Premere device can safely and effectively close PFO. Additional studies should be undertaken to demonstrate the effectiveness of PFO closure in reducing thrombo-embolic events such as stroke.

Is the Ross operation still an acceptable option in children and adolescents?

BACKGROUND: The Ross operation is increasingly accepted as an alternative to conventional valve prostheses for children, adolescents, and young adults. We review patients younger than 20 years of age. METHODS: Of 404 Ross operations done before November 2004, 60 were young patients with a median age of 12 years (range, 1 to 20 years). The pulmonary autograft technique universally was as a free root. A cryopreserved pulmonary homograft reconstructed the right ventricular outflow tract. RESULTS: Early postoperative complications were reentry for bleeding in 2 patients and one pacemaker insertion. No thromboembolic or hemorrhagic events occurred during the follow-up of 42 +/- 27 months. Two late deaths occurred, one from myocardial infarction after 3 months and another sudden death after 5 years, probably from critical pulmonary homograft stenosis. Echocardiographic follow-up revealed a median peak gradient of 6.3 +/- 3 mm Hg across the autograft. The median pulmonary homograft peak gradient of 19.1 +/- 13.7 mm Hg was increased to more than 30 mm Hg in 6 patients. Another 6 patients had moderate but clinically insignificant pulmonary homograft regurgitation. Altogether, 6 patients required reoperation for replacement of stenotic homografts. No autograft related reoperation occurred. CONCLUSIONS: Young patients with the Ross operation had good mid-term autograft function and no perioperative mortality. Factors that justify the choice of the Ross operation for young patients are the normal physiologic hemodynamics and growth of the autograft as well as freedom from anticoagulation. A 10% reoperation rate, elevated pulmonary homograft gradients, and the surgical complexity remain limiting factors.

Dural ectasia in children with Marfan syndrome: a prospective, multicenter, patient-control study.

UNLABELLED: The clinical diagnosis of Marfan syndrome in childhood is difficult, because symptoms may not have developed to their full expression until adulthood. The Ghent nosology for the diagnosis of Marfan syndrome classifies dural ectasia as a major diagnostic criterion. More than two thirds of adult patients with Marfan syndrome show dural ectasia, while the frequency in childhood is unknown. This prospective multicenter observational patient-control study was performed to identify pathologic changes of the lumbosacral spine in young patients with Marfan syndrome. DESIGN: Prospective clinical trial, multicentric, cross-sectional. SETTING: MRI of the lumbosacral spine. PATIENTS: Twenty patients with proven Marfan syndrome, 20 patients suspicious for Marfan syndrome and 38 healthy controls. OUTCOME MEASURES: Vertebral body diameter (VBD) from L1 to S1, dural sac diameter (DSD) from L1 to S1, dural sac ratio (DSR), qualitative assessment of the lumbosacral spine. RESULTS: DSD and VBD in different age groups were higher in patients with proven or suspected Marfan syndrome than in healthy controls (DSD: L1, 6-8 years, P < 0.05). VBD related to body height showed a similar growth related increase in patients with proven or suspected Marfan syndrome and controls. DSD related to body height was elevated in patients with proven or suspected Marfan syndrome at different levels of the lumbar spine. DSD at levels L1, L5, and S1, and DSR at levels L5 and S1 of patients with proven Marfan syndrome were significantly higher (P < 0.05) than in controls. CONCLUSION: Even during childhood pathologic changes inside the lumbosacral spine of patients with Marfan syndrome can be observed. Dural ectasia, which occurs at different levels of the lumbar spine, can be detected at levels L5 and S1 in up to 40% of patients with Marfan syndrome.

[Percutaneous procedures for the repair of iatrogenic occlusion or stenosis of peripheral vessels in children--report of 5 cases]. / Przezskórne zabiegi naprawcze zwezonych jatrogennie naczyn obwodowych. Opis pieciu przypadków.

Invasive percutaneous diagnostic or therapeutic procedures are associated with the risk of thrombosis and occlusion of peripheral vessels which are used for vascular access. Data on the transcatheter therapy of vascular complications in children are scarce. We described five children in four of whom percutaneous transluminal balloon angioplasty of occluded peripheral vessels was successfully performed. Technical aspects of this treatment and indications are discussed.

Clinical course and complications of infective endocarditis in patients growing up with congenital heart disease.

BACKGROUND: Although a high number of patients with congenital heart disease (CHD) undergo surgical palliation or definite correction up to adolescence, adult congenital heart disease (ACHD) may remain a potential lifelong risk factor for infective endocarditis (IE) in patients growing up with congenital heart disease (GUCH). METHODS: In a retrospective case study of a tertiary care center long-term clinical course and complications of patients with IE and GUCH were analysed. RESULTS: Data of 52 patients with CHD, who fulfilled the Saiman criteria for infective endocarditis and were treated between April 1986 and March 2001, were identified: Risk factors for infective endocarditis were previous cardiovascular operation (51.9%), use of foreign material (38.5%), dental or other surgical procedures without recommended antibiotic prophylaxis (25.0%), or cardiac catheterization (5.8%). Staphylococcal (38.9%) or streptococcal species (35.2%) were cultivated in most cases as causative microorganisms. Complications were: recurrence of IE (7.7%), septic embolisms (30.8%) leading to central nervous complications (7.7%), embolism of pulmonary arteries (7.7%), renal arteries (1.9%), arteries of the extremities (9.6%), or infarction of spleen (1.9%). Other cardiac (23.1%) or extracardiac (13.5%) complications were frequent. The need of re-operations during or after IE was high (67.3%). The hospital mortality was 1.9%, late mortality was 7.7%. CONCLUSIONS: Patients with IE and CHD show a broad clinical spectrum of cardiac and extracardiac complications. They may lead to a complicative short- and long-term course with the potential risk of death and a high number of re-operation. Efforts have to be made to improve long-term outcome of patients with ACHD by an interdisciplinary cooperation.

[Transluminal balloon valvuloplasty in neonates and infants with critical aortic stenosis]. / Transluminalna balonska valvuloplastika u novorodencadi i dojencadi s kriticnom aortnom stenozom.

The purpose of this study was to evaluate children who underwent balloon valvuloplasty due to critical aortic stenosis following clinical (low cardiac output, cardiogenic shock, congestive heart failure) and echocardiographic criteria (morphological evidence of left ventricular hypertrophy, with depression of left ventricular function, irrespective of transvalvular gradient). We assessed the effectiveness of balloon valvuloplasty in 5 children (all male) who were submitted to aortic valve balloon dilatation over 3.5 years (10.1998-05.2003). The age at dilatation was 29+/-24 days, BW 3.92+/-0.82 kg and BSA 0.24+/-0.03 m2. In all children the balloon valvuloplasty was performed with manual inflation of balloon at 4-6 bars through the femoral artery. The mean systolic pressure gradient across the aortic valve decreased from 68+/-20.5 mmHg to 9+/-10.95 mmHg, i.e. by 85%, (p<0.01). Aortic valve ring diameter was 9.2+0.84 mm, and balloon/aortic ring ratio 0.8-0.04. The degree of aortic insufficiency immediately after the dilatation did not significantly increase. Dilatation was performed without complications. Long term results were evaluated in all patients 3.2 - 54 months after valvuloplasty and revealed the continuously increasing residual aortic valve gradient (Doppler measurement) 33+/-10.95 mmHg to be significantly lower (p<0.01) than before valvuloplasty. None of the children was showing clinical symptoms of the disease. According to echocardiographic analysis two of them developed aortic valvar insufficiency grade II, two had trivial insufficiency, one was without insufficiency. One child is an candidate for the Ross procedure in future (gradient 50 mmHg, insufficiency grade II, age 4.5 years.). Balloon valvuloplasty provides effective interventional method in the treatment of the neonates and infants with critical aortic stenosis.