Abstract
Background
The thyroid gland of patients with congenital heart disease may be exposed to large doses of iodine from various sources. We assessed the thyroid response after iodine exposure ...during conventional angiography in cardiac catheterization and angiographic computer tomography in childhood.
Methods
Retrospective mid- to long-term follow-up of 104 individuals (24% neonates, 51% infants, 25% children) with a median age and body weight of 104 days 0–8 years and 5.3 kg 1.6–20. Serum levels of thyroid-stimulating hormone, free triiodthyronine and free thyroxine were evaluated at baseline and after excess iodine. We also assessed risk factors that may affect thyroid dysfunction.
Results
Baseline thyroidal levels were within normal range in all patients. The mean cumulative iodinate contrast load was 6.6 ± 1.6 mL/kg. In fact, 75% had experienced more than one event involving iodine exposure, whose median frequency was three times per patient 1–12. During the median three years follow-up period 0.5–10, the incidence of thyroid dysfunction was 15.4% (n=16). Those patients developed acquired hypothyroidism (transient n=14, long-lasting n=2 both died) with 10 of them requiring temporary replacement therapy for transient thyroid dysfunction, while four patients recovered spontaneously. 88 individuals (84.6%) remained euthyroid. Repeated cardiac interventions, use of drugs that interfere with the thyroid and treatment in the intensive care unit at the index date were strong predictors for acquired thyroid dysfunction.
Conclusions
The incidence of acquired hypothyroidism after iodine excess was 15.4%. However, most patients developed only transient hypothyroidism. Systemic iodine exposure seems to be clinically and metabolically well tolerated during long-term follow-up.
To assess the efficacy and safety of a breakable BabyStent to treat complex aortic coarctation (CoA) in early childhood. Although recommended in several guidelines, there is no approved aortic stent ...for young infants, because of the dilemma between two mandatory requirements: expandable up to adult size on the one hand, and small enough to fit through a baby's femoral artery on the other. Prospective interventional, multi‐center clinical trial with the breakable Osypka BabyStent® (OBS). The OBS is a low‐profile, 15‐mm long cobalt‐chromium stent, pre‐mounted on a 6 mm balloon and inserted via a 4 Fr sheath. After implantation, its diameter is adjustable from 6 to 12 mm by balloon dilation. Further dilation opens predefined joints enabling unrestricted growth. Nineteen patients (9 male), median age 112 days (range: 7–539), median body weight 5.6 kg (range: 2.4–8.4) were deemed high risk and underwent stent implantation. Of those, 74% suffered from re‐CoA following surgery, 53% had additional cardiac and 21% noncardiac malformations. Our primary combined endpoint was fulfilled: All stents were implanted in the desired region, and a >50% intrastenotic diameter‐extension was achieved in 15 patients (78.9%, 80% confidence interval 62.2; 90.5, 95% confidence interval 54.4; 93.9). Secondary endpoint confirmed that the OBS fits the baby's femoral vessel diameter. All children survived the procedure and 12‐month follow‐up. This stent enables percutaneous stenting of complex aortic coarctation to treat high‐risk newborns and infants.
The study describes our experience with Amplatzer Vascular Plugs (AVP2 and 4) and highlights a more refindes telescopic technique for AVP2 delivery. AVPs are well-established occlusion devices for ...vascular anomalies in congenital heart disease (CHD). The AVP2 is sometimes preferred to the AVP4 due to its shorter length, flat-profiled retention disks, and the availability of larger diameters, but its profile requires a larger inner lumen for safe delivery. The latter may actually hamper access to target lesions. This is a retrospective analysis of all CHD patients treated with the AVP2 and AVP4 between 12/2012 and 12/2015. Target vessels were characterized, measured, and the device-to-vessel diameter ratio calculated. A modified pigtail technique for AVP2 delivery was frequently used: a floppy wire was simply reinforced by the curved tip of a pigtail catheter (instead of the long sheath’s dilator) to guide the required delivery sheath towards the desired landing zone. 59 patients with a median age and bodyweight of 3.0 years (range 0.1–75) and 13.8 kg (range 2.5–80) underwent the implantation of 106 plug-devices (30 AVP2, 76 AVP4) in 91 target vessels. Indications for their use were ductus arteriosus (19%), aortopulmonary (43%) as well as venovenous collaterals (34%) and other miscellaneous lesions (4%). The pigtail-supported AVP2 delivery in six patients proved very convenient. No complications occurred. AVPs are excellent devices for embolizing shunt vessels in CHD patients. Here, we describe a simplified telescoping technique for AVP2 delivery to enter curvy target lesions gently and efficiently.
ObjectivesTo assess the potential occupational radiation reduction and technical feasibility in patients rotated 180° (upside-down) when requiring neck access for transcervical or trans-subclavian ...catheterisation.MethodsUpside-down positioning is defined as rotating patients in supine position by 180°, so that the feet come to rest where the head would otherwise be. We retrospectively evaluated all these procedures performed between March 2016 and May 2019. Furthermore, two different phantoms (paediatric and adult) were used prospectively to quantify the occupational dose between conventional or upside-down positioning. In this context, ambient dose equivalents were measured using real-time dosimeters. Three different projection angles were applied.Results44 patients with median age and body weight of 1.0 year (range 0–56) and 9.5 kg (range 1.3–74.3) underwent 63 procedures positioned upside-down. This position proved advantageous for practical reasons, since the length of the examination table could be optimally used. Additionally, it resulted in a significantly lower overall ambient dose equivalent for the primary operator (PO) of 94.8% (mean: 2569±807 vs 135±23 nSv; p<0.01) in the adult, and of 65.5% (mean: 351±104 vs 121±56 nSv; p<0.01) in the paediatric phantom, respectively.ConclusionUpside-down positioning facilitates handling in a straightforward manner when access from the neck is required. Moreover, it significantly reduces local radiation exposure for the PO in the paediatric and, most impressively, in the adult phantom.
Aneurysms of the azygos vein Kreibich, Maximilian, MD; Siepe, Matthias, MD; Grohmann, Jochen, MD ...
Journal of vascular surgery. Venous and lymphatic disorders (New York, NY),
07/2017, Letnik:
5, Številka:
4
Journal Article
Abstract Objective Azygos vein aneurysms (AVAs) are uncommon and infrequently diagnosed. When confronted with a patient presenting with an AVA, physicians can rely on only a few case reports after an ...extensive literature search. To date, no guideline, no rule, and no review on the optimal treatment strategy for these patients exist. Methods A PubMed and MEDLINE database search for papers and case reports describing AVA was performed. Cases from our own institutions were also reviewed. Results The literature search identified 57 published case reports that were reviewed for inclusion. Of those published cases, etiologic factors can be classified into idiopathic, acquired, and traumatic causes. Most AVAs are limited to the azygos arch, a congenital anatomic weak point. Clinical symptoms generally remain nonspecific. Computed or magnetic resonance tomography scans are effective diagnostic tools, although the optimal therapeutic plan remains unclear. Complications include rupture, thromboembolism, mediastinal mass effects, and pulmonary artery hypertension. Conclusions Conservative treatment along with oral anticoagulation may be reasonable for some AVAs, but to date, there is no clear guideline or evidence-based threshold for surgical or interventional therapy. In review of the existing data and from our clinical and scientific knowledge, interventional or surgical treatment should strongly be considered in cases with clinical symptoms, pulmonary embolism or pulmonary arterial hypertension, thrombus formation within the AVA in patients with oral anticoagulation or for patients with a contraindication to oral anticoagulants, considerable increase in diameter or compression of adjacent structures, saccular AVA, or an underlying connective tissue disease. The most common procedure is surgical ligation of the AVA, although endovascular occlusion of the aneurysms is becoming more frequent.
Aortic valve regurgitation leading to coronary steal phenomenon can severely impair cardiac function in hypoplastic left heart syndrome, thus worsening long-term outcome.
A German infant with ...borderline aortic and mitral valve, hypoplastic left ventricle, ventricular septal defect, and hypoplastic aortic arch with critical coarctation initially underwent aortic arch reconstruction and aortic valve dilation with the aim of biventricular correction later on. Unfortunately, severe cardiac dysfunction necessitated a change in strategy entailing modified stage I Norwood palliation. Increasing aortic regurgitation with coronary steal was revealed postoperatively, which required redo surgery to oversew the valve. However, pronounced aortic regurgitation recurred, causing severe cardiac decompensation with repeated resuscitation. As a bailout strategy, we performed aortic valve closure via transfemoral retrograde implantation of an Amplatzer Duct Occluder II device. This led to the patient's rapid stabilization while circumventing highly risky renewed surgery in such a critically ill infant.
Retrograde transcatheter aortic valve closure may be considered a feasible alternative in infants with a failing single ventricle due to aortic regurgitation, with critical device evaluation being crucial for successful device implantation in this young age group.
Congenital extrahepatic portosystemic shunts (CEPS), previously also described as Abernethy malformations, are rare malformations in which the extrahepatic portal system directly communicates with ...the vena cava inferior, thereby bypassing the liver. A hypoplastic portal vein (PV) exists in most cases. CEPS have been associated with the development of liver nodules, ranging from mostly focal nodular hyperplasia (FNH) to hepatic adenoma (HA) and even hepatocellular carcinoma (HCC). Tumor development in CEPS may be due to changes in perfusion pressures, oxygen supply or endocrine imbalances. It is important to rule out CEPS in children with liver tumors, because resection could impede future shunt occlusion procedures, and benign masses may regress after shunt occlusion. Here, we review the case of a 9-years-old male with CEPS and hepatic nuclear Factor 1-alpha (HNF-1-alpha) inactivated HA to raise awareness of this condition and review histopathological changes in the liver of CEPS.
Aberrant right subclavian artery(arteria lusoria) is the most common congenital root anomaly, remaining asymptomatic in most cases. Nevertheless, some of the 20%-40% of those affected present ...tracheo-esophageal symptoms. We report on a 6-year-old previously healthy girl presenting with progressive dysphagia over 4 wk. Diagnostics including barium swallow, echocardiography and magnetic resonance angiography(MRA) revealed a retro-esophageal compression by an aberrant right subclavian artery. Despite the successful, uneventful transposition of this arteria lusoria to the right common carotid via right-sided thoracotomy, the girl was suffering from persisting dysphagia. Another barium swallow showed the persistent compression of the esophagus on the level where the arteria lusoria had originated. As MRA showed no evidence of a significant re-obstruction by the transected vascular stump, we suspected a persisting ligamentum arteriosum. After a second surgical intervention via left-sided thoracotomy consisting of transecting the obviously persisting ligamentum and shortening the remaining arterial stump of the aberrant right subclavian artery, the patient recovered fully. In this case report we discuss the potential relevance of a persisting ligamentum arteriosum for patients with leftaortic arch suffering from dysphagia lusoria and rational means of diagnosing, as well as the surgical options to prevent re-do surgery.
Aneurysms of the pulmonary artery Kreibich, Maximilian; Siepe, Matthias; Kroll, Johannes ...
Circulation (New York, N.Y.),
01/2015, Letnik:
131, Številka:
3
Journal Article
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