scholarly journals Az aortaív-anomáliák klinikai jelentősége a magzattól a felnőttkorig

2021 ◽  
Vol 162 (48) ◽  
pp. 1920-1923

Összefoglaló. Az intrauterin echokardiográfiás és MR-diagnosztika fejlődése napjainkban jelentős szerepet tulajdonít már az első trimeszter idejében kimutatható aortaív-fejlődési rendellenességeknek. Célunk volt részletezni a vascularis gyűrűk megjelenési formáit, ezek különböző életkorokban jelentkező tüneteit, sebészi kezelését, hogy hozzájárulhassunk a helyes felvilágosításhoz, mely alapvető lehet a szülés helyének megválasztásában, így meghatározhassák a magzat sorsát, perinatalis ellátását. A situs inversustól eltekintve a jobb oldali aortaív jelenléte mindig felhívja a figyelmet vascularis gyűrű, társuló szívhibák, genetikai betegségek, kromoszómaanomáliák jelenlétére, tehát a magzat további vizsgálata szükséges. Kettős aortaív esetén jelentkezhetnek olyan súlyos tünetek, melyek a megszületés után, kora csecsemőkorban sebészi beavatkozást igényelhetnek (szükséges lehet a szülés helyének megválasztása!). Az aberráns jobb arteria subclavia önállóan nem alkot érgyűrűt, a ritkán társuló truncus caroticusszal csak későbbi életkorban okozhat sebészi beavatkozást igényló enyhe tüneteket. Orv Hetil. 2021; 162(48): 1920–1923. Summary. Nowadays, the development of intrauterine echocardiography and MR diagnostics plays a significant role in aortic arch malformations detected during the first trimester. Our aim was to detail the manifestations of vascular rings, their symptoms at different ages and their surgical treatment options in order to determine the fate of the fetus and its perinatal care. Apart from situs inversus, the presence of the right aortic arch always draws the attention to the possible presence of vascular rings, associated heart defects, genetic diseases, or chromosomal abnormalities, therefore further examinations of the fetus are necessary. In the case of a double aortic arch, severe symptoms may occur, which may require surgery after birth and in early infancy (it may be necessary to choose the place of birth!). The right aberrant subclavian artery does not form a ring and may cause mild symptoms requiring surgery at a later age with rarely associated truncus carotid. Orv Hetil. 2021; 162(48): 1920–1923.

2020 ◽  
Vol 21 (Supplement_1) ◽  
Author(s):  
I Campos ◽  
C Vieira ◽  
N Salome ◽  
V H Pereira ◽  
A Costeira Pereira ◽  
...  

Abstract Introduction Complete vascular rings represent about 0.5-2% of all congenital cardiovascular malformations, with the double aortic arch (DAA) being the most common of the complete vascular rings, causing tracheoesophageal compression. The right (posterior) arch is usually dominant (70%), although the two arches can have the same size (5%). The left (anterior) arch is dominant in only approximately 25% of cases. In most cases, this anomaly is diagnosed during childhood due to symptoms caused by oesophageal or tracheal compression. For this reason, case reports of adults are rare. This report describes a case of a 61-year-old woman with DAA with dominant left arch, diagnosed accidentallyby thoracic CT angiography. Case Report Description A 61 years old woman with a previous story of hypertension and type 1 diabetes presented to the emergency service with dyspnoea and thoracic pain. She also referred a history of intermittent dysphagia and cough with at least 12 years of progression. All the parameters of the physical examination were within normal limits. The electrocardiogram showed a normal sinus rhythm with no evidence of acute ischemia and her blood analyses did not show any abnormalitie. She also performed a thoracic CT angiography, which excluded signs of pulmonary embolism, but revealed a vascular ring suggesting a double aortic arch with permeability in both right and left arches as well as their collaterals. The Cardiac MRI was performed with the purpose of excluding ischemia, confirming the double aortic arch with left dominance. The right arch, posterior to the oesophagus and trachea, and the left arch, in an anterior position, showed an anatomic compression of the oesophagus as well as the proximal trachea, capable of eliciting the symptoms mentioned. Other congenital anomalies were excluded. The echocardiography did not demonstrate any additional cardiac malformation. Endoscopy shows a pulsatile extrinsic compression of the esophagus (aortic ring). The patient is currently being studied and closely monitored in the Cardiology consultation. Discussion The most common type of complete vascular ring is the double aortic arch, which accounts for 70% of the complete rings. In most cases, there are two permeable arches, usually with right dominance (70% of the cases). Rarely, both arches are symmetrical. Symptoms usually appear in the fifth month of life. In most cases, only supportive treatment is required. Conclusion This case illustrates the atypical features of this congenital malformation, namely the diagnosis during adulthood as well as the left dominance. Abstract P725 Figure. A double aortic arch


Author(s):  
O.L. Galkina, N.A. Savel'eva, I.V. Poddubnaya

During 5 years in the first trimester of pregnancy 82 cases of congenital anomalies of fetal development were diagnosed, in 14 cases (17%) an omphalocele was found. An analysis of the results of prenatal examination of fetuses with omphalocele in an isolated and combined version has been carried out. In 78% of cases, omphalocele was combined with other fetal anomalies, most often with congenital heart defects (82%). The frequency of chromosomal abnormalities in fetuses with omphalocele was 69%. Perinatal outcome in the case of early prenatal omphalocele diagnosis is unfavorable in 93% of cases. Early prenatal diagnosis of omphalocele is possible in 100% of cases. Diagnosed in the first trimester of pregnancy, omphalocele is an indication for an extended anatomical evaluation of the fetus with the aim of forming a clinical hypothesis and conducting timely optimal prenatal counseling.


2018 ◽  
Vol 24 (3) ◽  
pp. 157-160
Author(s):  
Hălmaciu Ioana ◽  
Suciu Bogdan Andrei ◽  
Roşca Sorin ◽  
Nagy Bota Monica Cristina ◽  
Trâmbiţaş Cristian ◽  
...  

Abstract Introduction. Double aortic arch (DAA) represents a vascular malformation generated by the persistence of the right dorsal aorta from the intrauterine life. An aortic ring is formed, that surrounds the trachea and esophagus, resulting in difficulty in breathing and swallowing. Case report. We report the case of a 13 years old male child who was admitted to the pediatric surgery department accusing the presence of a paravertebral subcutaneous lesions. Histopathological result of the excised lesion revealed the presence of peripheral T-cell lymphoma. Examination of computer tomography angiography (Angio-CT) revealed the presence of a complete arterial chain (aortic double arch - DAA) around the trachea and esophagus, without signs of compression. Most cases are diagnosed in the first year of life, the literature reports a few cases of DAA diagnosed late, to the adolescent or adult. Clinically most of the anatomical variants are usually symptomatic especially that are associated with congenital heart defects, including also Fallot tetralogy. Conclusions. Angio-CT is a very useful method in diagnosing arterial or venous malformations, symptomatic or asymptomatic.


Ultrasound ◽  
2021 ◽  
pp. 1742271X2110572
Author(s):  
Rachel Annetta ◽  
Debbie Nisbet ◽  
Edward O’Mahony ◽  
Ricardo Palma-Dias

Introduction The right subclavian artery normally arises as the first vessel from the brachiocephalic trunk. An aberrant right subclavian artery (ARSA) arises directly from the aortic arch and crosses behind the trachea towards the right arm. This variant occurs in approximately 1–2% of the population; however, the frequency increases in individuals with chromosomal abnormalities such as trisomy 21 and 22q11.2 microdeletion. Prenatal identification of ARSA therefore has a role in screening for such conditions. Methods Databases were searched for studies reporting the prenatal ultrasound evaluation of ARSA and its frequency in normal fetuses and in those with chromosomal abnormalities. Results A total of 23 studies were evaluated. Feasibility for the ultrasound evaluation of ARSA was 85–95%. The sonographic detection of ARSA is best in the three-vessel trachea view; however, sagittal and coronal imaging of the aortic arch may be useful. ARSA in isolation was not found to be associated with chromosomal abnormalities. The prevalence of ARSA in chromosomally abnormal fetuses was up to 24-fold higher than in normal fetuses, but the majority of chromosomally abnormal fetuses with ARSA had additional abnormal ultrasound findings, particularly cardiac abnormalities. Conclusions The prenatal detection of ARSA is a clinically useful prenatal marker for chromosomal abnormalities. In isolation, it is unlikely to be associated with pathogenic genetic variants. The ultrasound diagnosis of ARSA should prompt meticulous assessment of associated abnormalities. Invasive diagnostic testing should be offered to patients with non-isolated ARSA or in the presence of non-reassuring screening results or other risk factors.


2019 ◽  
Vol 18 (1) ◽  
pp. 5-11
Author(s):  
Aleksandra Łukasiewicz ◽  
Barbara Baranowska ◽  
Edyta Dzierżak-Postek ◽  
Piotr Węgrzyn

AbstractIntroduction. The standard of perinatal care gives the woman the right to consciously choose the place of birth. Birth Centres (BC) run by midwives implement a vision of a non-medicalized birth in intimate homelike conditions.Aim. The aim of the study is to determine the causes of transfers from the Birth Centre to the Obstetric Unit and to indicate the social and demographic characteristics of the women giving birth that may predispose to transfer.Material and methods. The study was based on the analysis of the medical records of women who were transferred from the BC to the St. Sophia Obstetric Unit in Warsaw. 88 women who were transferred constituted the study group.Results. The most numerous group were women giving birth aged 30-34. The majority were giving birth for the first time and had higher education. Intrapartum transfers were related to the mother’s obstetric condition, fetal condition or disruption of the childbirth.Conclusions. Failure to progress of labour is the main cause of transfers. The women giving birth for the first time aged 30 and over, admitted for to the BC after 40 weeks of pregnancy and dilation below 4 cm were the most often transferred group of women.


Author(s):  
Е.А. Калашникова ◽  
Е.Н. Андреева ◽  
П.А. Голошубов ◽  
Н.О. Одегова ◽  
Е.В. Юдина ◽  
...  

В ходе анализа результатов раннего пренатального скрининга (РПС) в России за 2018 г. (Аудит-2019) дана оценка качества мероприятий, общей эффективности и тенденций развития системы РПС в субъектах РФ посредством сравнения рассчитанных основных организационных, методологических и интегральных показателей с международными референтными значениями. In the course of analyzing the results of early prenatal combined first-trimester screening (FTS) in Russia for 2018 (Audit-2019) the assessment of the quality of measures, the overall effectiveness and trends in the development of the FTS system in the regions of Russia. They are presented by comparing the calculated main organizational, methodological and integral indicators with international reference values.


2020 ◽  
pp. 13-17
Author(s):  
Dmitrii Aleksandrovich Lopyn ◽  
Stanislav Valerevich Rybchynskyi ◽  
Dmitrii Evgenevich Volkov

Currently the electrophysiological treatment options have been considered to be the most effective for many patients with arrhythmogenic cardiomyopathies, as well as in those with arrhythmias on the background of heart failure. Currently, the dependence of efficiency of the pacemakers on the location of the electrodes has been proven. In order to study the effect of a myocardial dysynchrony on the effectiveness of pacing depending on the location of the right ventricular electrode, an investigation has been performed. This study comprised the patients with a complete atrioventricular block, preserved ejection fraction of the left ventricle (more than 50 %), with no history of myocardial infarction, who were implanted with the two−chamber pacemaker. It has been established that the best results were achieved with a stimulation of the middle and lower septal zone of the right ventricle, the worst ones were obtained with a stimulation of its apex. It has been found that the dynamics of the magnitude of segmental strains and a global longitudinal strain coincided with the dynamics of other parameters of the pacemaker effectiveness, which indicated the pathogenetic value of myocardial dysynchrony in the progression of heart failure after implantation of the pacemaker. Therefore it could be concluded that the studying of myocardial mobility by determining a longitudinal strain for assessing the functional state of the myocardium and the effectiveness of pacing is highly advisable. It is emphasized that the use of the latest strains−dependent techniques for cardiac performance evaluation in the patients with bradyarrhythmia have a great potential to predict the development of chronic heart failure and to choose the optimal method of physiological stimulation of the heart. Key words: right ventricular lead, cardiac stimulation, myocardial dyssynchrony.


Author(s):  
I.V. Novikova, O.M. Khurs, T.V. Demidovich et all

16 second trimester fetuses with 22q11.2 deletion syndrome have been examined at anatomic-pathological investigation. Main cardiovascular diseases were ascending aorta hypoplasia with aortic valve stenosis (n = 6; 37.5%), truncus arteriosus (n = 5; 31.25%), tetralogy of Fallot (n = 3; 18.75%) and double-outlet right ventricle (n = 1; 6.25%). Ventricular septal defect was present in 16 cases. Associated aortic arch anomalies included interrupted aortic arch (n = 9; 56.25%), right aortic arch (n = 6; 37.5%), retroesophageal ring (n = 1; 6.25%) and aberrant right subclavian arteria (n = 5; 31.25%). 5 fetuses had left ventricular outflow tract obstructive lesions with interrupted aortic arch of type B combined with aberrant right subclavian arteria.


2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Alessandro Robaldo ◽  
Guido Carignano ◽  
Alberto Balderi ◽  
Claudio Novali

Management of the symptomatic multiple stenosis of supra-aortic vessels (MSSVs) in a “bovine” aortic arch (BAA) configuration is infrequently reported. The optimal treatment choice remains debatable. A successful hybrid treatment for a proximal critical stenosis of the innominate and left common carotid artery was performed in a high-risk patient with a tandem symptomatic lesion in the right carotid bifurcation and a concentric vulnerable plaque in the bovine trunk. This case supports the feasibility, safety, and efficacy of a combined carotid bifurcation endarterectomy and retrograde kissing stenting of common carotid arteries with cerebral protection after evaluation of radiological, anatomical, and clinical parameters.


2021 ◽  
pp. 1-2
Author(s):  
Niall Linnane ◽  
Andrew Green ◽  
Colin J. McMahon

Abstract 16p12.2 microdeletion has been associated with congenital heart defects and developmental delay. In this case, we describe the rare association between tetralogy of Fallot with an absent pulmonary valve a right-sided aortic arch and a retro-aortic innominate vein associated with a 16p12.2 microdeletion and epilepsy.


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