Az aortaív-anomáliák klinikai jelentősége a magzattól a felnőttkorig

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.

Mid-term results of vascular ring surgery

Background: Vascular rings cause respiratory symptoms in children. Treatment consists of surgical division; however, data regarding mid-term results are scarce. The purpose of this study was to evaluate clinical results of vascular ring surgery. Methods: Retrospective chart review of consecutive patients who underwent vascular ring surgery. Mid-term follow-up consisted of clinic visits and telephone questionnaire over a 1-year period sampling at five points in time. Results: Follow-up of 85 patients who underwent vascular rings surgery revealed significant symptomatic improvement within 6 months. In total, 50% were symptomatic to some degree at their last documented clinic visit complaining of stridor (36.8%), cough (34.2%), wheezing (10.5%), dyspnoea on exertion (10.5%), or recurrent respiratory infections (23.7%). By telephone questionnaires, 83% described a significant improvement in respiratory symptoms, 63.4% had some residual symptoms, 36.6% stridor, 38.8% chronic cough, 32.4% bronchodilator use,18.3% had at least one episode of pneumonia per year, 31% of children had dyspnoea or respiratory limitation, and 12.7% while doing physical activities. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Conclusions: Surgical division of vascular rings results in significant clinical improvement within 1 year; nevertheless, some patients remain symptomatic to some degree. We found no association between the age at surgery or anatomic variant to the presence of symptoms at mid-term follow-up. Further evaluation is warranted to assess the nature of residual symptoms and explore whether anatomical causes can be identified leading to surgical modifications.

SURGICAL MANAGEMENT OF COMPLETE VASCULAR RINGS: OUR EXPERIENCE

Vascular rings are rare congenital aortic arch anomalies that may cause trachea-esophageal compressive symptoms in infants and children. It may be asymptomatic in few cases. Due to its rare occurrence and non-specic symptoms, the diagnosis is often missed, thus delaying the treatment. With this case series we share our experience of managing four cases of vascular rings over four years, with age of patients ranging from 3 months to 3 years. All cases presented in pediatric emergency with trachea-esophageal compressive symptoms. In our series we encountered 4 types of complete vascular rings- Left Pulmonary Artery (LPA) sling with Left Ligamentum (LL), Right Aortic Arch with aberrant Left Subclavian Artery from Kommerell diverticulum with LL, Right dominant Double Aortic Arch (DAA) with associated TOF, and DAA with left dominant arch. Two cases had associated cardiac anomalies, which were Atrial Septal Defect (ASD) and Tetralogy Of Fallot (TOF). Median sternotomy was done in two cases with associated cardiac anomalies and left thoracotomy was the approach for rest two cases. Single stage single incision approach was followed in all cases, with no postoperative mortality and no signicant morbidity. Early diagnosis and precise planning of surgical intervention provides the best long-term outcomes.

Airway obstruction in children with complex conotruncal cardiac anomalies

The incidence of airway obstruction in patients with complex CHD other than vascular rings and absent pulmonary valve syndrome is unknown. We reviewed pre-operative CT and clinical data of children with conotruncal abnormalities to assess for airway obstruction. Airway obstruction was common (41% of patients), often moderate to severe, of diverse aetiology, and most commonly associated with a right aortic arch. Patients with airway obstruction showed a trend towards a higher mortality rate. Patients with complex conotruncal abnormalities should be assessed for airway obstruction as it may help predict the need for additional interventions and assist with prognostication.