Carney complex featuring giant intracardiac biatrial myxoma in an adolescent girl Get access Ventsislav Sheytanov, Ventsislav Sheytanov Sana Kliniken AG, Cardiac Surgery, Herdweg 2, Stuttgart, Germany Search for other works by this author on: Oxford Academic PubMed Google Scholar Heike Schaefer, Heike Schaefer Sana Kliniken AG, Cardiac Surgery, Herdweg 2, Stuttgart, Germany Search for other works by this author on: Oxford Academic PubMed Google Scholar Peter Hitzle, Peter Hitzle Sana Kliniken AG, Cardiac Surgery, Herdweg 2, Stuttgart, Germany Search for other works by this author on: Oxford Academic PubMed Google Scholar Robert Nossal, Robert Nossal Klinikum Stuttgart, Olgahospital, Kriegsbergstraße 62, Stuttgart, Germany Search for other works by this author on: Oxford Academic PubMed Google Scholar Frank Uhlemann, Frank Uhlemann Klinikum Stuttgart, Olgahospital, Kriegsbergstraße 62, Stuttgart, Germany Search for other works by this author on: Oxford Academic PubMed Google Scholar Iannis Tzanavaros, Iannis Tzanavaros Sana Kliniken AG, Cardiac Surgery, Herdweg 2, Stuttgart, Germany Search for other works by this author on: Oxford Academic PubMed Google Scholar Fabian Spoehr, Fabian Spoehr Sana Kliniken AG, Cardiac Surgery, Herdweg 2, Stuttgart, Germany Search for other works by this author on: Oxford Academic PubMed Google Scholar Joerg Seeburger Joerg Seeburger Sana Kliniken AG, Cardiac Surgery, Herdweg 2, Stuttgart, Germany Corresponding author. Tel: +49-711-278-36009; Email: joerg.seeburger@sana.de https://orcid.org/0000-0003-0292-1519 Search for other works by this author on: Oxford Academic PubMed Google Scholar European Heart Journal, Volume 42, Issue 44, 21 November 2021, Page 4605, https://doi.org/10.1093/eurheartj/ehaa1047 Published: 28 December 2020 Article history Received: 02 December 2020 Editorial decision: 07 December 2020 Published: 28 December 2020 Corrected and typeset: 20 November 2021

Das 10-jährige türkischstämmige Mädchen wurde aus einer auswärtigen Klinik übernommen mit hohem Fieber seit 2 Wochen sowie heftigen Bauchschmerzen. Es war eine Appendektomie durchgeführt worden, mikroskopisch zeigte die Appendix eine lymphofollikuläre Invasion mit der histologischen Diagnose einer katarrhalischen Appendizitis. Intraoperativ wurde eine ausgeprägte Lymphadenitis mesenterialis mit Enteritis und Aszites beschrieben. Zu den weiterhin persistierenden Bauchschmerzen klagte das Mädchen über ausgeprägte multitope Myalgien und Arthralgien. Im Alter von 3 Jahren war eine Purpura Schönlein-Henoch (PSH) durchgemacht worden. 2 Wochen vor der Erkrankung trat eine Rachenentzündung ohne Erregernachweis auf.

A Solysafe septal occluder (Swissimplant AG, Solothurn, Switzerland) had been implanted in a 41-year-old woman with an ostium secundum atrial septal defect (ASD). A regular flat configuration of the device with some residual shunting was documented immediately after implantation. Nineteen months

Overall and regardless of the technique used for the repair of complete AVSD, good early and midterm outcomes were achieved.

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 (TM) stent system for newborns and infants is a L605 cobalt-chromium, 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 (4-6mm). It offers a new, unique stent design that allows post-dilation steps up to empty set11.5 mm. While re-dilating up to empty set11.5 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.gov NCT03287024). Conclusion: The low-profile BeGrow (TM) 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. (c) 2018 The Authors. Published by Elsevier B.V.

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.

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.

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 (Delta P-max) across the RVOT Elan conduit were 15 +/- 3.2; 15.3 +/- 2.1 Delta, 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.

Percutaneous pulmonary valve implantation (PPVI) has emerged to the preferred mode of treatment for right ventricular outflow tact (RVOT) valved conduit dysfunction in some centers1, 2. While homografts and distensible heterograft conduits are usually dilatable to larger diameters, even if considerably shrunk and calcified, non-distensible Dacron- or PTFE-conduits and mounted bioprostheses cannot be augmented in diameter. So far in these patients surgical replacement is the only treatment option if the diameters of the implants are too small. We report on in vitro conceptional testing followed by catheter interventional treatment (PPVI) of three patients with a stenotic Edwards Perimount bioprosthesis (Edwards Lifesciences Corporation, Irvine, US) in the RVOT. Erratum to “Cracking the ring of Edwards Perimount bioprosthesis with ultrahigh pressure balloons prior to transcatheter valve in valve implantation” [Int. J. Cardiol. 176 (2014) 1048–1049]International Journal of CardiologyVol. 197PreviewThe correct affiliations of the authors are the following: Full-Text PDF

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.

Zielsetzung: Für die Behandlung von Stenosen in kindlichen Gefäßen mit Wachstumspotential existiert derzeit keine optimale Lösung. Der hier vorgestellte Kobalt-Chrom-Stent der Firma Bentley Innomed GmbH erlaubt bei einem initialen Implantationsdurchmesser von 5-6mm (4F Schleuse) ein Nachdilatieren entsprechend dem Gefäßwachstum bis 12mm Durchmesser unter Erhalt der Integrität des Stents ohne Verkürzung und ein Öffnen des Stents entlang einer zirkulären Sollbruchstelle bei Dilatation über diesen Durchmesser hinaus. Damit ist die Option einer langfristigen Behandlung von Stenosen bis ins Erwachsenenalter gegeben.

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.

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.

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.

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.

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.